Long-term sequential deferiprone-deferoxamine versus deferiprone alone for thalassaemia major patients: a randomized clinical trial

DEFF Research Database (Denmark)

Maggio, Aurelio; Vitrano, Angela; Capra, Marcello

2009-01-01

A multicentre randomized open-label trial was designed to assess the effectiveness of long-term sequential deferiprone-deferoxamine (DFO-DFP) versus DFP alone to treat thalassaemia major (TM). DFP at 75 mg/kg, divided into three oral daily doses, for 4 d/week and DFO by subcutaneous infusion (8-1...

New trend in the epidemiology of thalassaemia.

Science.gov (United States)

Li, Chi-Kong

2017-02-01

Thalassaemia is the most common monogenic disorder worldwide. It is common in areas with prevalent malaria as thalassaemic red cells provide immunity against the parasite. The incidence of thalassaemia carriers is high in regions such as Mediterranean, Middle East, Indian subcontinent, Southeast Asia and South China. In the past few decades, migrants from the thalassaemia prevalent countries to non-prevalent countries, mainly North America and Central and North Europe, are rapidly increasing in number. The non-prevalent countries may not have established pre-natal screening system for thalassaemia. The genetic subtypes among the different ethnic groups vary; this may pose challenges in prenatal diagnosis. Genetic counselling on the postnatal course of thalassaemia may be affected by the genotype-phenotype correlation and coinheritance of other genetic diseases. New treatment methods improve the survival of patient with thalassaemia major, but some late complications that occur with longer survival have been recently discovered. Copyright © 2016. Published by Elsevier Ltd.

Psychological therapies for thalassaemia.

Science.gov (United States)

Anie, Kofi A; Massaglia, Pia

2014-03-06

Thalassaemia is a group of genetic blood disorders characterised by the absence or reduction in the production of haemoglobin. Severity is variable from less severe anaemia, through thalassaemia intermedia, to profound severe anaemia (thalassaemia major). In thalassaemia major other complications include growth retardation, bone deformation, and enlarged spleen. Blood transfusion is required to treat severe forms of thalassaemia, but this results in excessive accumulation of iron in the body (iron overload), removed mostly by a drug called desferrioxamine through 'chelation therapy'. Non-routine treatments are bone marrow transplantation (which is age restricted), and possibly hydroxyurea, designed to raise foetal haemoglobin level, thus reducing anaemia. In addition, psychological therapies seem appropriate to improving outcome and adherence to medical treatment. To examine the evidence that in people with thalassaemia, psychological treatments improve the ability to cope with the condition, and improve both medical and psychosocial outcomes. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Haemoglobinopathies Trials Register which comprises of references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Searches on the Internet were also performed.Date of the most recent search of the Group's Haemoglobinopathies Trials Register: 11 November 2013. All randomised or quasi-randomised controlled trials comparing the use of psychological intervention to no (psychological) intervention in people with thalassaemia. No trials of psychological therapies have been found in the literature for inclusion in this review. There are currently no results to be reported. As a chronic disease with a considerable role for self-management, psychological support seems appropriate for managing thalassaemia. However, from the information currently available, no conclusions

THALASSAEMIA INTERMEDIA : AN UPDATE

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Maria Domenica Cappellini

2009-06-01

Full Text Available

Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with thalassaemia intermedia (TI has substantially increased over the past decade. TI encompasses a wide clinical spectrum of beta-thalassaemia phenotypes. Some TI patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with TI are rarely seen in thalassaemia major, including extramedullary hematopoiesis, leg ulcers, gallstones, thrombosis and pulmonary hypertension. There are a number of options currently available for managing patients with TI, including transfusion therapy, iron chelation therapy, modulation of foetal haemoglobin production and haematopoietic stem cell transplantation. However, at present, there are no clear guidelines for an orchestrated optimal treatment plan.

Anaesthesia for a patient with B-thalassaemia

African Journals Online (AJOL)

population, but there is scarce information regarding β-thalassaemia major in adults. With current medical ... blood pressure was 80/55 mm Hg with sinus rhythm at 80 bpm, .... paid to each one of the systems involved in order to achieve the.

Effect of nutrition support on immunity in paediatric patients with beta-thalassaemia major.

Science.gov (United States)

Tienboon, Prasong

2003-01-01

Nutritional deficiencies have been variably observed in thalassaemia and the aetiology of many of the immune abnormalities in thalassaemic children are poorly defined. Therefore, we tested the hypothesis that certain immune abnormalities have a nutritional basis. Nutritional status, selective quantitative and functional indices of immunity were studied in twelve children (7 females, 5 males; mean age 28 months, SD 5 and range 19.8-35.5), with thalassaemia major before and after a one month period of intensive nutrition support (the study diet consisted of 'Enfapro' liquid formula (Mead Johnson) with added dextrose and corn oil to achieve a caloric density of 1.1 kcal/cc in addition to vitamins and minerals). Each child was provided approximately 150 kcal/day and 4 g of protein/day. Lymphocyte proliferation to Concanavalin A (Con A) (P = 0.008) and Purified Protein Derivative (PPD) (P = 0.002) was depressed upon entry into the study, however the response to Con A attained normal values by the end of the intervention. Compared to baselines, the proliferative response to Con A (P = 0.005) and Phytohemagglutinin A (PHA) (P = 0.031) both improved after the nutrition support. Although there was no general correlation of zinc status with lymphocyte proliferation, normal baseline zinc status was associated with improvement of proliferation. The %CD4 increased (P = 0.036), primarily because of a decrease in total lymphocytes and to lesser extent a decrease in CD8 lymphocytes. Serum immunoglobulin concentrations were found to be elevated on admission but were not significantly affected by the nutrition intervention. C3 concentrations were uniformly depressed on admission but increased by the end of the study protocol (P = 0.037). C4 and CH50 activity were not significantly influenced by the intervention. In conclusion, children with beta thalassaemia have abnormalities of lymphocyte function as well as key complement components that are responsive to nutrition support. In

Osteoporosis Syndrome in Thalassaemia Major: An Overview

Directory of Open Access Journals (Sweden)

Meropi Toumba

2010-01-01

Full Text Available Osteoporosis in thalassaemia major (TM represents a prominent cause of morbidity. The mechanism of pathogenesis of bone disease (BD in TM is multifactorial and complicated. Peak bone mass is achieved shortly after completion of puberty and normally remains stable until the third decade of life when age-related bone mass begins. Growth hormone (GH and sex steroids play a crucial role in bone remodeling and in the maintenance of skeletal architecture during adult life. GH and insulin growth factors (IGFs have anabolic effect in bone formation. Sex steroids act probably by increasing the expression of RANKL by osteoblastic cells and alterations in the RANK/RANKL/OPG system in favor of osteoclasts. Impaired GH secretion and lack of sex steroids in thalassemic patients due to pituitary damage, contribute to failure of achieving optimal peak bone mass. Other endocrine complications such as hypoparathyroidism and vitamin D deficiency have also a detrimental role on bones in TM. It is still questionable whether the international criteria for defining osteopenia and osteoporosis are relevant to patients with TM; also a question arises for the diagnostic methods such as DEXA scan and management of osteoporosis with known treatment protocols, in the thalassaemic patient.

Acute liver failure in a patient with sickle cell/β+ thalassaemia

International Nuclear Information System (INIS)

Wigg, A.J.; Mounkley, A.D.; Cochlan, D.; Somers, S.

2001-01-01

We describe a rare, severe, vaso-occlusive presentation of sickle cell disease, named sickle cell intrahepatic cholestasis (SCIC). Patients with sickle cell/β + thalassaemia frequently have mild vaso-occlusive symptoms and only one case of SCIC developing in a patient with sickle cell/β + thalassaemia has been previously described in the world literature. The present report represents only the second described case of SCIC in a patient with sickle cell/β + thalassaemia. An abdominal computed tomography scan and Doppler ultrasound studies demonstrated massive hepatomegaly (25 cm span). Liver biopsy was performed and demonstrated dilatation and congestion of erythrocytes, severe cholestasis and fibrosis. The case demonstrates the importance of early recognition and institution of adequate therapy. Initial and correct diagnosis does not require biopsy or surgery which carry substantial risks of bleeding and mortality

Dual energy x-ray absorptiometry (DEXA) in the assessment of liver iron in patients with beta thalassaemia major

International Nuclear Information System (INIS)

Chatterton, B.E.; Thomas, C.M.; Schultz, C.G.

2000-01-01

Full text: Beta thalassaemia major is a condition in which anaemia from abnormal haemoglobin production causes bone marrow expansion and frequently reduced bone mineral density. These patients have a chronic requirement for transfusion which results in tissue iron overload which may cause organ damage. Increased X-ray attenuation in the liver was noted in patients undergoing whole body DEXA for the assessment of bone density and it was assumed that this was related to liver iron stores. The aim of this study was to determine if useful information about liver iron could be obtained from these studies. Method: Using a Lunar DPXL, whole body scanning was performed in 16 patients (eight male) age 19-32 with Beta Thalassaemia. As well as calculating indices of total body composition, regions of interest were placed over the visualised liver. The 'bone mineral content' (BMC),g and bone mineral density (BMD),g/cm 2 were calculated over the liver regions, with the assumption that the calculation related to mineral in the region of interest. The results were compared with the serum ferritin as an indirect measure of body iron stores. Results showed a highly significant correlation (r=0.85) between 'BMD' in the liver region and ferritin. Conclusion: Despite the known difficulties with equating iron stores and ferritin, and possible confounders on liver density, such as fibrosis, the high correlation suggests that DEXA may have a place in the assessment of iron deposition, and be more cost effective than other technologies such as MRI and CT. Prospective studies with invasive measurements of liver iron will be needed to determine this. Copyright (2000) The Australian and New Zealand Society of Nuclear Medicine Inc

Sample acceptance time criteria, electronic issue and alloimmunisation in thalassaemia.

Science.gov (United States)

Trompeter, S; Baxter, L; McBrearty, M; Zatkya, E; Porter, J

2015-12-01

To determine the safety of a 1-week acceptance criteria of sample receipt in laboratory to transfusion commencement in transfusion dependent thalassaemia with respect to alloimmunisation. To determine the safety of electronic issue of blood components in such a setting. Retrospective audit of alloimmunisation (1999-2012) and blood exposure in registered thalassaemia patients at a central London thalassaemia centre where the acceptance criteria for the group and save sample from arrival in the laboratory to the time of issue of blood for transfusion for someone who has been transfused in the last 28 days was 1 week, and there was electronic issue protocol for patients who have always had a negative antibody screen (other than temporary positivity in pregnant women receiving prophylactic anti-D or anti Le-a, Anti Le-b and Anti P1 that are no longer detectable). There were 133 patients with thalassemia variants regularly attending UCLH for review. A total of 105 patients had transfusion dependent thalassaemia (TDT) (7 E-beta thalassaemia, 98 beta thalassaemia major). Ten of the 84 patients who received their transfusions at UCLH were alloimmunised. Seven of them had been alloimmunised prior to arrival at UCLH. Only two patients developed antibodies at UCLH during this period. The prevalence of alloantibody formation of 2% in UCLH transfused patients, with presumptive incidence of 0.01 alloantibodies per 100 units or 0·001 immunisations per person per year compares favourably with other reported series and suggests that 1 week interval with appropriate electronic issue is acceptable practice. © 2015 British Blood Transfusion Society.

Internal distribution of excess iron and sources of serum ferritin in patients with thalassaemia

International Nuclear Information System (INIS)

Cazzola, M.; Bergamaschi, G.; Dezza, L.; Borgna-Pignatti, C.C.; De Stefano, P.; Bongo, I.G.; Avato, F.

1983-01-01

Liver and spleen iron concentrations, serum ferritin level and binding of S-ferritin to concanavalin A (Con A) were measured in 12 patients with thalassaemia major or intermedia at the time of splenectomy. All these subjects had increased liver iron concentration, most of them had hepatic fibrosis but none of them had histological evidence of chronic hepatitis. No patient had ascorbic acid deficiency. Serum ferritin concentration was increased in all cases, ranging from 266 to 5504 μg/l. In all but 2 subjects most of the protein did not bind to Con A, thus behaving as tissue ferritin. There were highly significant correlations between serum ferritin concentration, amount of blood transfused and liver iron concentration. On the avarage, iron concentration in the liver was about 3 times that in the spleen. The findings obtained suggest that in patients with thalassaemia major or intermedia most of the iron is deposited in parenchymal tissues and most of the S-ferritin derives by leakage from the cytosol of iron-loaded parenchymal cells. S-ferritin is a valid index of liver iron overload in thalassaemic patients witout complications such as viral hepatitis and/or ascorbic acid defiency. (author)

Delta-Beta Thalassaemia in a Pathan Family.

Science.gov (United States)

Ahmad, Saqib Qayyum; Zafar, Saerah Iffat; Malik, Hamid Saeed; Ahmed, Suhaib

2017-11-01

Delta-beta-thalassaemia (δβ-thalassaemia) is a rare type of thalassaemia which mostly results from deletion of δ and β genes with preservation of γ genes. δβ-thalassaemia is classified into (δβ)+ and (δβ)0 types. The (δβ)0-thalassemia is further divided into GγAγ(δβ)0-thalassaemia and Gγ(Aγδβ)0-thalassaemia. In heterozygous state, (δβ)0mutations give rise to phenotype resembling β-thalassaemia trait but with raised Hb-F, ranging from 5 to 20%, without a rise in Hb-A2. In homozygotes, the clinical picture is usually that of thalassaemia intermedia and the patients have 100% Hb-F. Workup of a 1-year child suffering from pallor, chronic ill health, and splenomegaly referred to our laboratory with the suspicion of β-thalassaemia, ultimately resulted in a diagnosis on polymerase chain reaction as having homozygous inversion/deletion Gγ(Aγδβ)0-thalassaemia. Her family members were also investigated.

Expression of CD55, CD59, and CD35 on red blood cells of β-thalassaemia patients

Science.gov (United States)

Koçtekin, Belkls; Kurtoǧlu, Erdal; Yildiz, Mustafa; Bozkurt, Selen

2017-01-01

Aim of the study β-thalassaemia (β-Thal) is considered a severe, progressive haemolytic anaemia, which needs regular blood transfusions for life expectancy. Complement-mediated erythrocyte destruction can cause both intravascular and extravascular haemolysis. Complement regulatory proteins protect cells from such effects of the complement system. We aimed to perform quantitative analysis of membrane-bound complement regulators, CD55 (decay accelerating factor – DAF), CD35 (complement receptor type 1 – CR1), and CD59 (membrane attack complex inhibitory factor – MACIF) on peripheral red blood cells by flow cytometry. Material and methods The present study was carried out on 47 β-thalassemia major (β-TM) patients, 20 β-thalassaemia intermedia (β-TI) patients, and 17 healthy volunteers as control subjects. Results CD55 levels of β-TM patients (58.64 ±17.06%) were significantly decreased compared to β-TI patients (83.34 ±13.82%) and healthy controls (88.57 ±11.69%) (p < 0.01). CD59 levels of β-TM patients were not significantly different than β-TI patients and controls, but CD35 levels were significantly lower in the β-TM patients (3.56 ±4.87%) and β-TI patients (12.48 ±9.19%) than in the control group (39.98 ±15.01%) (p < 0.01). Conclusions Low levels of CD55 and CD35 in thalassaemia major patients indicates a role for them in the aetiopathogenesis of haemolysis in this disease, and also this defect in a complement system may be responsible for the chronic complications seen in these patients. PMID:28680334

Alpha-thalassaemia and response to hydroxyurea in sickle cell anaemia.

Science.gov (United States)

Darbari, Deepika S; Nouraie, Mehdi; Taylor, James G; Brugnara, Carlo; Castro, Oswaldo; Ballas, Samir K

2014-04-01

Hydroxyurea (HU) reduces vaso-occlusive crises (VOC) and other complications of sickle cell anaemia (SCA). Alpha-thalassaemia is a known modifier of SCA. Studies on the efficacy of HU in SCA patients with α-thalassaemia have yielded varying results. To determine the effect of α-thalassaemia in response to HU therapy in the Multicenter Study of Hydroxyurea (MSH) cohort. We compared the laboratory parameters and VOC incidence in the MSH cohort stratified by the presence or the absence of α-thalassaemia. Hydroxyurea showed significant (P = 0.001 for all baseline vs. follow-up comparisons) treatment effect on red cell indices irrespective of α-globin gene deletion. The magnitude of the HU-related changes was similar for mean corpuscular volume (MCV) (no α-thalassaemia 13 fl and α-thalassaemia 13 fl) and mean corpuscular haemoglobin (MCH) (no α-thalassaemia 4 pg and α-thalassaemia 4 pg) in both groups. Foetal haemoglobin (HbF) and F-cells also increased significantly with HU treatment in both groups. Total haemoglobin increased after HU treatment in both groups, but the increase was smaller and not statistically significant in patients with α-thalassaemia. In contrast, HU-related reduction in VOCs was more pronounced in patients with α-thalassaemia (VOC incidence rate ratio HU/placebo: 0.63 for α-thalassaemia and 0.54 for no α-thalassaemia (P for interaction 0.003). Hydroxyurea decreases VOCs in SCA patients with and without α-thalassaemia, and the degree of VOC reduction was more pronounced in the patients with alpha-thalassaemia. Despite the lower baseline values, changes in standard laboratory parameters such as MCV and HbF percent remain useful in monitoring HU therapy in the presence of α-thalassaemia. © 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

α-thalassaemia

Directory of Open Access Journals (Sweden)

Higgs Douglas R

2010-05-01

. Carriers of alpha+- or alpha0-thalassaemia alleles generally do not need treatment. HbH patients may require intermittent transfusion therapy especially during intercurrent illness. Most pregnancies in which the foetus is known to have the haemoglobin Bart's hydrops foetalis syndrome are terminated due to the increased risk of both maternal and foetal morbidity.

MRI evaluation of tissue iron burden in patients with {beta}-thalassaemia major

Energy Technology Data Exchange (ETDEWEB)

Argyropoulou, Maria I.; Astrakas, Loukas [University of Ioannina, Radiology Department, Medical School, Ioannina (Greece)

2007-12-15

{beta}-Thalassaemia major is a hereditary haemolytic anaemia that is treated with multiple blood transfusions. A major complication of this treatment is iron overload, which leads to cell death and organ dysfunction. Chelation therapy, used for iron elimination, requires effective monitoring of the body burden of iron, for which serum ferritin levels and liver iron content measured in liver biopsies are used as markers, but are not reliable. MRI based on iron-induced T2 relaxation enhancement can be used for the evaluation of tissue siderosis. Various MR protocols using signal intensity ratio and mainstream relaxometry methods have been used, sometimes with discrepant results. Relaxometry methods using multiple echoes achieve better sampling of the time domain in which relaxation mechanisms take place and lead to more precise results. In several studies the MRI parameters of liver siderosis have failed to correlate with those of other affected organs, underlining the necessity for MRI iron evaluation in individual organs. Most studies have included children in the evaluated population, but MRI data on very young children are lacking. Wider application of relaxometry methods is indicated, with the establishment of universally accepted MRI protocols, and further studies, including young children, are needed. (orig.)

A time-driven, activity-based costing methodology for determining the costs of red blood cell transfusion in patients with beta thalassaemia major.

Science.gov (United States)

Burns, K E; Haysom, H E; Higgins, A M; Waters, N; Tahiri, R; Rushford, K; Dunstan, T; Saxby, K; Kaplan, Z; Chunilal, S; McQuilten, Z K; Wood, E M

2018-04-10

To describe the methodology to estimate the total cost of administration of a single unit of red blood cells (RBC) in adults with beta thalassaemia major in an Australian specialist haemoglobinopathy centre. Beta thalassaemia major is a genetic disorder of haemoglobin associated with multiple end-organ complications and typically requiring lifelong RBC transfusion therapy. New therapeutic agents are becoming available based on advances in understanding of the disorder and its consequences. Assessment of the true total cost of transfusion, incorporating both product and activity costs, is required in order to evaluate the benefits and costs of these new therapies. We describe the bottom-up, time-driven, activity-based costing methodology used to develop process maps to provide a step-by-step outline of the entire transfusion pathway. Detailed flowcharts for each process are described. Direct observations and timing of the process maps document all activities, resources, staff, equipment and consumables in detail. The analysis will include costs associated with performing these processes, including resources and consumables. Sensitivity analyses will be performed to determine the impact of different staffing levels, timings and probabilities associated with performing different tasks. Thirty-one process maps have been developed, with over 600 individual activities requiring multiple timings. These will be used for future detailed cost analyses. Detailed process maps using bottom-up, time-driven, activity-based costing for determining the cost of RBC transfusion in thalassaemia major have been developed. These could be adapted for wider use to understand and compare the costs and complexities of transfusion in other settings. © 2018 British Blood Transfusion Society.

Hydroxyurea responses in clinically varied beta, HbE-beta thalassaemia and sickle cell anaemia patients of Eastern India.

Science.gov (United States)

Chatterjee, Tridip; Chakravarty, Amit; Chakravarty, Sudipa

2018-05-01

The haematological and clinical response to hydroxyurea was estimated in HbE-beta, beta thalassaemia and sickle cell anaemia patients of Eastern India, with variable clinical severity and transfusion requirement to determine whether hydroxyurea can help these patients to maintain their steady haemoglobin level without blood transfusions. Three hundred patients (189 HbE-beta thalassaemia, 95 beta thalassaemia and 16 other haemoglobinopathies including sickle cell anaemia) were selected for hydroxyurea therapy and were followed up for 48-60 months. Results suggest significant response to hydroxyurea therapy in 19 beta and 99 HbE-beta patients in the transfusion-dependent group (GR-I). All of them became transfusion-independent while on hydroxyurea therapy. The majority of responding patients were IVS1-5(G-C) in one of their alleles in HbE-beta cases (83 out of 119). Though IVS1-5(G-C) was found to be the commonest mutation in our selected patients, the mutational background of the patients does not found to have any significant correlation with the response category towards hydroxyurea as per the results observed in our study. But, the drug works pretty well in most of the transfusion-dependent patients, as these patients were withdrawn from regular blood transfusion. At the same time, partial or no response to the drug hydroxyurea was also recorded in our study.

Beta-thalassaemia trait: haematological parameters

International Nuclear Information System (INIS)

Yousafzai, Y.M.; Khan, S.; Raziq, F.

2010-01-01

Thalassaemia syndromes are a group of hereditary disorders characterised by a genetic deficiency in the synthesis of --globin genes. The objective of this study was to determine the haematological features -thalassaemia trait (BTT), and to determine the sensitivity of Mean Corpuscular Volume (MCV), Mean Corpuscular Haemoglobin (MCH) and -thalassaemia trait. Methods: A descriptive study was conducted in Hayatabad Medical Complex, Peshawar from May 2009 to May 2010 with 203 subjects having BTT. Blood samples were collected in EDTA anti-coagulated tubes. RBC indices were taken as part of complete blood count (CBC) by haematology analyser, and Haemoglobin (Hb) electrophoresis was done to determine the HbA2 percentage. The data was collected and analyzed on statistical software for demographic details, RBC indices and HBA2 levels. Results: Out of 203 patients, 92 (45%) were males and 111 (55%) were females. Most patients tested were in the 15-45 year age group. One-hundred-sixty (79%) patients had anaemia. MCV was lower than 76 fl in all the cases. Mean MCV was 59.1 fl. MCH was low, the mean MCH being 19.3 g/dl. MCH <26 gave sensitivity of 99% in detecting BTT. We calculated MI for these cases and found out that it was <12 in 75% of cases and <15 in 197 (97%). Conclusion: Beta-thalassaemia traits present with a microcytic hypochromic blood picture, detected on simple haematology analysers as low MCV and MCH and MI which provide a beta- thalassaemia trait. (author)

Relative protection from ischaemic heart disease in beta-thalassaemia carriers

International Nuclear Information System (INIS)

Bozdar, M.; Ahmed, S.; Anwar, J.

2010-01-01

To compare the frequency of beta thalassaemia trait in individuals with Ischaemic Heart Disease (IHD) and a control population without IHD. Study Design: Case control study. Place and Duration of Study: Department of Haematology, Armed Forces Institute of Pathology (AFIP), Rawalpindi, from September 2007 to May 2009. Methodology: Using non-probability consecutive sampling, a total of 544 subjects were selected, including 272 IHD patients and an equal number of age and gender matched normal controls. The subjects were tested for the presence of b-thalassaemia trait by performing their blood counts, haemoglobin electrophoresis and Haemoglobin A2 (HbA2) estimation. Proportions were compared using chi-square test. Odds ratio was also calculated. Results: The frequency of b-thalassaemia trait was determined in IHD patients and was compared to the frequency in normal Pakistani population. Six out of the 272 control subjects (2.2%) had b-thalassaemia trait and one of the control subject had Haemoglobin D trait. In contrast, none of the 272 IHD patients had b-thalassaemia trait. The calculated odds ratio was less than 1, which shows a significant negative association of b-thalassaemia trait with IHD. The difference in the frequency of b-thalassaemia trait in the two groups was statistically significant (p=0.033). Conclusion: The results suggest that b-thalassaemia carriers have some protection against IHD, though it is not an absolute cardio protection due to the role of other risk factors in IHD. This beneficial information may be communicated to the concerned individuals in their counselling sessions and as part of general awareness on thalassaemia. (author)

Public perceptions and attitudes toward thalassaemia: Influencing factors in a multi-racial population.

Science.gov (United States)

Wong, Li Ping; George, Elizabeth; Tan, Jin-Ai Mary Anne

2011-03-30

Thalassaemia is a common public health problem in Malaysia and about 4.5 to 6% of the Malays and Chinese are carriers of this genetic disorder. The major forms of thalassaemia result in death in utero of affected foetuses (α-thalassaemia) or life-long blood transfusions for survival in β-thalassaemia. This study, the first nationwide population based survey of thalassaemia in Malaysia, aimed to determine differences in public awareness, perceptions and attitudes toward thalassaemia in the multi-racial population in Malaysia. A cross-sectional computer-assisted telephone interview survey of a representative sample of multi-racial Malaysians aged 18 years and above was conducted between July and December 2009. Of a total of 3723 responding households, 2846 (76.4%) have heard of thalassaemia. Mean knowledge score was 11.85 (SD ± 4.03), out of a maximum of 21, with higher scores indicating better knowledge. Statistically significant differences (P culturally acceptable in the reduction of pregnancies with thalassaemia major. The findings provide insights into culturally congruent educational interventions to reach out diverse socio-demographic and ethnic communities to increase knowledge and cultivate positive attitudes toward prevention of thalassaemia.

Molecular characteristic of alpha thalassaemia among patients diagnosed in UKM Medical Centre.

Science.gov (United States)

Azma, Raja Zahratul; Ainoon, Othman; Hafiza, Alauddin; Azlin, Ithnin; Noor Farisah, Abudul Razak; Nor Hidayati, Sardi; Noor Hamidah, Hussin

2014-04-01

Alpha (Α) thalassaemia is the most common inherited disorder in Malaysia. The clinical severity is dependant on the number of Α genes involved. Full blood count (FBC) and haemoglobin (Hb) analysis using either gel electrophoresis, high performance liquid chromatography (HPLC) or capillary zone electrophoresis (CE) are unable to detect definitively alpha thalassaemia carriers. Definitive diagnosis of Α-thalassaemias requires molecular analysis and methods of detecting both common deletional and non-deletional molecular abnormailities are easily performed in any laboratory involved in molecular diagnostics. We carried out a retrospective analysis of 1623 cases referred to our laboratory in Universiti Kebangsaan Malaysia Medical Centre (UKMMC) for the diagnosis of Α-thalassaemia during the period October 2001 to December 2012. We examined the frequency of different types of alpha gene abnormalities and their haematologic features. Molecular diagnosis was made using a combination of multiplex polymerase reaction (PCR) and real time PCR to detect deletional and non-deletional alpha genes relevant to southeast Asian population. Genetic analysis confirmed the diagnosis of Α-thalassaemias in 736 cases. Majority of the cases were Chinese (53.1%) followed by Malays (44.2%), and Indians (2.7%). The most common gene abnormality was ΑΑ/--(SEA) (64.0%) followed by ΑΑ/-Α(3.7) (19.8%), -Α(3.7) /--(SEA) (6.9%), ΑΑ/ΑΑCS (3.0%), --(SEA)/--(SEA) (1.2%), -Α(3.7)/-Α(3.7) (1.1%), ΑΑ/-Α(4.2) (0.7%), -Α(4.2)/--(SEA (0.7%), -Α(3.7)/-Α(4.2) (0.5%), ΑΑ(CS)/-- SEA) (0.4%), ΑΑ(CS)/ΑΑ(Cd59) (0.4%), ΑΑ(CS)/ΑΑ(CS) (0.4%), -Α(3.7)/ΑΑ(Cd59) (0.3%), ΑΑ/ΑΑ(Cd59) (0.1%), ΑΑ(Cd59)/ ΑΑ(IVS I-1) (0.1%), -Α(3.7)/ΑΑ(CS) (0.1%) and --(SEA) /ΑΑ(Cd59) (0.1%). This data indicates that the molecular abnormalities of Α-thalassaemia in the Malaysian population is heterogenous. Although Α-gene deletion is the most common cause, non-deletional Α-gene abnormalities

Beta thalassaemia traits in Nigerian patients with sickle cell anaemia ...

African Journals Online (AJOL)

Journal of Medicine and Biomedical Research ... These three patients (1.2%) were found to have positive co-inheritance of thalassaemia trait and sickle cell anaemia. The erythrocyte indices were all reduced in these selected families except for one family whose mean cell haemoglobin concentration was within normal ...

Thalassaemia Trait with Gaucher Disease: A Diagnostic Dilemma.

Science.gov (United States)

Kini, Jyoti Ramnath; Sreeram, Saraswathy; Hegde, Anupama; Kamath, Sowmini; Pai, Radha Ramachandra

2017-09-01

Gaucher Disease is an autosomal recessive disease caused by the accumulation of glucocerebrosidase due to deficiency in lysosomal glucocerebrosidase. Thalassaemia trait is asymptomatic and is usually an incidental diagnosis. Both thalassaemia and Gaucher disease can have similar haematologic manifestations and hence, their coexistence causes diagnostic dilemma. Our patient presented at one-and-a-half years with weakness, pallor, failure to thrive and massive hepatosplenomegaly. Clinical examination and history pointed to a lipid storage disease. Peripheral smear revealed microcytic hypochromic cells and nucleated red cells with haemolytic blood picture. Thalassaemia trait was indicated on haemoglobin variant analysis using High Performance Liquid Chromatography. Liver biopsy, bone marrow aspirate and therapeutic splenectomy revealed Gaucher-like cells. Type 1 Gaucher disease can be clinically asymptomatic as well as present with massive liver and spleen enlargement and involvement of bone marrow. Anaemia, splenomegaly and thrombocytopenia are the usual presentations at diagnosis, similar to the haemoglobinopathies. Gaucher-like cells with normal beta-glucocerebrosidase (pseudo-Gaucher cells) are seen in thalassaemia, leukaemia, mycobacterial infections and myeloma. Gaucher disease coexisting with thalassaemia trait is uncommon. We report the occurrence of thalassaemia trait and Gaucher disease in a child, which resulted in confusion regarding the haematological diagnosis. This report highlights the necessity of independent establishment of the diagnosis in every patient so that appropriate management decisions are taken.

Alpha thalassaemia-mental retardation, X linked

Directory of Open Access Journals (Sweden)

Gibbons Richard

2006-05-01

Full Text Available Abstract X-linked alpha thalassaemia mental retardation (ATR-X syndrome in males is associated with profound developmental delay, facial dysmorphism, genital abnormalities and alpha thalassaemia. Female carriers are usually physically and intellectually normal. So far, 168 patients have been reported. Language is usually very limited. Seizures occur in about one third of the cases. While many patients are affectionate with their caregivers, some exhibit autistic-like behaviour. Patients present with facial hypotonia and a characteristic mouth. Genital abnormalities are observed in 80% of children and range from undescended testes to ambiguous genitalia. Alpha-thalassaemia is not always present. This syndrome is X-linked recessive and results from mutations in the ATRX gene. This gene encodes the widely expressed ATRX protein. ATRX mutations cause diverse changes in the pattern of DNA methylation at heterochromatic loci but it is not yet known whether this is responsible for the clinical phenotype. The diagnosis can be established by detection of alpha thalassaemia, identification of ATRX gene mutations, ATRX protein studies and X-inactivation studies. Genetic counselling can be offered to families. Management is multidisciplinary: young children must be carefully monitored for gastro-oesophageal reflux as it may cause death. A number of individuals with ATR-X are fit and well in their 30s and 40s.

Thalassaemia and pregnancy

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Luk'yanenko, Viktoriya; Droogh, Marjoes; Overgaard, Ulrik Malthe

2017-01-01

Global migration has resulted in a larger geographical spread of people with risk of hereditary anaemias. This leads to an increased incidence of pregnant women with rare diseases, including thalassaemia also in Scandinavia. Thalassaemia can cause severe anaemia and other complications during pre...... pregnancy, like risk of miscarriage, intrauterine fetal death, abruptio, intrauterine growth retardation, hypertension, gestational diabetes and pre-eclampsia. In this article, we focus on the aetiology, assessment, antenatal care and treatment of pregnant women with thalassaemia....

Thalassaemia and pregnancy

DEFF Research Database (Denmark)

Luk'yanenko, Viktoriya; Droogh, Marjoes; Overgaard, Ulrik Malthe

2017-01-01

Global migration has resulted in a larger geographical spread of people with risk of hereditary anaemias. This leads to an increased incidence of pregnant women with rare diseases, including thalassaemia also in Scandinavia. Thalassaemia can cause severe anaemia and other complications during...... pregnancy, like risk of miscarriage, intrauterine fetal death, abruptio, intrauterine growth retardation, hypertension, gestational diabetes and pre-eclampsia. In this article, we focus on the aetiology, assessment, antenatal care and treatment of pregnant women with thalassaemia....

Assessment and management of iron overload in β-thalassaemia major patients during the 21st century: a real-life experience from the Italian WEBTHAL project.

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Piga, Antonio; Longo, Filomena; Musallam, Khaled M; Cappellini, Maria Domenica; Forni, Gian Luca; Quarta, Giovanni; Chiavilli, Francesco; Commendatore, Francesca; Mulas, Sergio; Caruso, Vincenzo; Galanello, Renzo

2013-06-01

We conducted a cross-sectional study on 924 β-thalassaemia major patients (mean age 30·1 years) treated at nine Italian centres using the WEBTHAL software, to evaluate real-life application of iron overload assessment and management standards. Serum ferritin 2 years. Patients who never had a cardiac MRI (CMR) T2* measurement were 2 years. Deferoxamine (22·8%) was more commonly used in patients with Hepatitis C Virus or high serum creatinine. Deferiprone (20·6%) was less commonly prescribed in patients with elevated alanine aminotransferase; while a deferoxamine + deferiprone combination (17·9%) was more commonly used in patients with serum ferritin >2500 ng/ml or CMR T2* <20 ms. Deferasirox (38·3%) was more commonly prescribed in patients <18 years, but less commonly used in those with heart disease or high iron intake. These observations largely echoed guidelines at the time, although some practices are expected to change in light of evolving evidence. © 2013 John Wiley & Sons Ltd.

Haematological and genetic features of delta beta-thalassaemia in Pakistan

International Nuclear Information System (INIS)

Ahmad, S.; Anwar, M.

2006-01-01

Objective: To describe the hematological and genetic features of delta beta-thalassaemia in Pakistani patients. Design: Descriptive case series. Place and Duration of Study: Department of Pathology, PNS Shifa, Karachi and Department of Hematology, Armed Forces Institute of Pathology, Rawalpindi, from January 1994 to April 2004. Patients and Methods: Thirteen individuals from six unrelated Pakistani families with a hematological diagnosis of delta beta-thalassaemia were studied. A brief clinical history, and the results of blood counts, absolute values, Hb-F, Hb-A/sub 2/, and hemoglobin electrophoresis were recorded. The DNA from each subject was first screened for the delta beta-thalassaemia mutations found in the Pakistani population. The samples were then screened for the Invl Del sup G/gamma(sup A/gamma delta beta). Results: The subjects included six heterozygote, six homozygotes and one compound heterozygote of delta beta and delta beta-thalassaemia. All heterozygote and 4/6 homozygotes were asymptomatic. One homo zygote had thalassaemia intermedia while another had transfusion dependent anemia. The mean Hb, TRBC, MCV, MCH, Hb-F and Hb-A/sub 2/ in delta beta-thalassaemia heterozygote were 11.6 g/dl, 5.37 x 1012/L, 70.9 fl, and 21.7 pg, 14% and 2.6% respectively. The same values in the four un transfused homo zygote were 10.6 g/dl, 5.34x1012/L, 69.211, and 20.8pg, 100% and 0% respectively. The mutation analysis revealed that all 13 individuals had the same Invl Del sup G/gamma(sup A/gamma delta beta). Conclusion: delta beta-thalassaemia is a rare disorder in Pakistan. Although the clinical picture is very mild its combination with delta beta-thalassaemia trait can produce a sever transfusion dependent thalassaemia. The DNA based diagnosis is possible in the prenatal as well as the postnatal period. (author)

The role of OMICS research in understanding phenotype variation in thalassaemia: the THALAMOSS project

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Roberto Gambari

2014-12-01

Full Text Available The β-thalassaemias are a group of severe and rare anaemias with monogenic inheritance, a complex systemic phenotype and several treatment-related complications, caused by more than 300 mutations of the β-globin gene. Novel therapeutic protocols, most of which are based on still experimental treatments, show great promise but significant variability of success between patients. These strategies include chemical/ molecular induction of the endogenous β-like g-globin gene or the restoration of clinically relevant β-globin levels by gene therapy. A small number of modifiers with significant impact on disease penetrance, severity and efficacy of treatments are known, but most remain elusive. Improvements of existing treatment regimens and optimization and application of novel treatments will critically depend on the characterization of additional disease modifiers and the stratification of patients for customized treatment regimens. This requires extensive analyses based on “OMICS”, an English-language neologism which refer to different but connected fields in molecular biology and biochemistry, such as genomics, transcriptomics, exomics, proteomics, metabolomics. The major objective of OMICS is a collective characterization of pools of biological molecules (gene sequences, transcripts, proteins and protein domains controlling biological structures, functions and dynamics, including several involved in pathological conditions. One of the most interesting observations of genomics in β-thalassaemias is the association between genomic sequences and high fetal haemoglobin (HbF levels, in consideration of the fact that high HbF levels are usually associated with milder forms of β-thalassaemia. Related to this issue, is the possibility to predict response to different therapeutic protocols on the basis of genomic analyses. For instance, three major loci (Xmn1-HBG2 single nucleotide polymorphism, HBS1L-MYB intergenic region on chromosome 6q, and

Cost effectiveness of once-daily oral chelation therapy with deferasirox versus infusional deferoxamine in transfusion-dependent thalassaemia patients: US healthcare system perspective.

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Delea, Thomas E; Sofrygin, Oleg; Thomas, Simu K; Baladi, Jean-Francois; Phatak, Pradyumna D; Coates, Thomas D

2007-01-01

Deferasirox is a recently approved once-daily oral iron chelator that has been shown to reduce liver iron concentrations and serum ferritin levels to a similar extent as infusional deferoxamine. To determine the cost effectiveness of deferasirox versus deferoxamine in patients with beta-thalassaemia major from a US healthcare system perspective. A Markov model was used to estimate the total additional lifetime costs and QALYs gained with deferasirox versus deferoxamine in patients with beta-thalassaemia major and chronic iron overload from blood transfusions. Patients were assumed to be 3 years of age at initiation of chelation therapy and to receive prescribed dosages of deferasirox and deferoxamine that have been shown to be similarly effective in such patients. Compliance with chelation therapy and probabilities of iron overload-related cardiac disease and death by degree of compliance were estimated using data from published studies. Costs ($US, year 2006 values) of deferoxamine administration and iron overload-related cardiac disease were based on analyses of health insurance claims of transfusion-dependent thalassaemia patients. Utilities were based on a study of patient preferences for oral versus infusional chelation therapy, as well as published literature. Probabilistic and deterministic sensitivity analyses were employed to examine the robustness of the results to key assumptions. Deferasirox resulted in a gain of 4.5 QALYs per patient at an additional expected lifetime cost of $US126,018 per patient; the cost per QALY gained was $US28,255. The cost effectiveness of deferasirox versus deferoxamine was sensitive to the estimated costs of deferoxamine administration and the quality-of-life benefit associated with oral versus infusional therapy. Cost effectiveness was also relatively sensitive to the equivalent daily dose of deferasirox, and the unit costs of deferasirox and deferoxamine, and was more favourable in younger patients. Results of this analysis

Genetic counselling in the beta-thalassaemias

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Adonis S. Ioannides

2013-03-01

Full Text Available The beta-thalassaemias are very important genetic disorders of haemoglobin synthesis and are amongst the commonest monogenic disorders. In view of the severity of beta-thalassaemia major, a number of screening programmes have been developed aimed at reducing the number of individuals born with the condition. Genetic counsellingplays a vital role in this process supporting the successful implementation of screening and delineating available options to at risk individuals. This review assesses the contribution of genetic counsellingat each stage of this process in the context of new diagnostic techniques and therapeutic options and discusses some of the more challenging aspects such as genotype/ phenotype correlation and coinheritance of other genetic conditions or genetic modifiers.

Prevalence of diabetes mellitus in Chinese children with thalassaemia major.

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Liang, Yuzhen; Bajoria, Rekha; Jiang, Yan; Su, Hongwei; Pan, Hongfei; Xia, Ning; Chatterjee, Ratna; Lai, Yongrong

2017-06-01

Diabetes mellitus is a common endocrinopathy in patients with β-thalassaemia major (β-TM), which is high prevalent in southern China. This study aimed to determine the cause and prevalence of glycaemic disorders in Chinese children with β-TM. In this prospective study, fasting glucose and insulin (FINS) levels were assessed in 267 β-TM and 80 non-TM control children. Homeostatic model assessment (HOMA) and the quantitative insulin sensitivity check index (QUICKI) were evaluated. Iron overload was assessed by serum ferritin (SF), total units of blood transfused and cardiac T2*. β-TM had higher FPG (P 10 years (OR 6.5; 95% CI 3.7-11.4; P 2500 μg/l (OR 4.8; 95% CI 2.1-11.1; P 50 IU/l (OR 2.1; 95% CI 1.2-3.7; P < 0.05) and cardiac T2* of <20 ms (OR 3.3; 95% CI 1.7-6.6; P < 0. 01). The children on deferiprone (DFP) had a reduced incidence of glycaemic aberrations than those on other chelating agents (OR 0.4; 95% CI 0.23-0.8; P < 0.05). Our data suggest that IFG occurred in 30% of β TM children, perhaps due to insulin resistance secondary to iron overload. Deferiprone-containing chelating agent may have a protective effect. © 2017 The Authors. Tropical Medicine & International Health Published by John Wiley & Sons Ltd.

HbA2 levels in β-thalassaemia carriers with the Filipino β0-deletion: are the levels higher than what is found with non-deletional forms of β0-thalassaemia?

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George, E; Teh, Lai Kuan; Tan, Jama; Lai, Mei I; Wong, Lily

2013-01-01

Classical carriers of β-thalassaemia are identified by a raised HbA2 level. Earlier studies indicated that the Filipino β-deletion has high raised HbA2 levels. The introduction of automated high performance liquid chromatography (HPLC) for thalassaemia screening is an important advance in technology for haematology laboratories. The BioRad Variant II Hb analyser is a common instrument used to quantify HbA2 levels in thalassaemia screening. This study aimed to determine HbA2 levels in carriers of Filipino β-mutation using the BioRad Variant II Hb analyser. The Filipino β-deletion was identified using gap-polymerase chain reaction (PCR) in the parents of transfusion dependent β-thalassaemia patients who were homozygous for the Filipino β-deletion in the indigenous population of Sabah, Malaysia. Hb subtypes were quantified on the BioRad Variant II Hb analyser. Concurrent α-thalassaemia was identified by multiplex gap-PCR for deletions and amplification refractory mutation system (ARMS)-PCR for non-deletional mutations. The mean HbA2 level for Filipino β-thalassaemia trait was 5.9 ± 0.47 and with coinheritance of α-thalassaemia was 6.3 ± 0.44 (-α heterozygous) and 6.7 ± 0.36 (-α homozygous). The HbA2 levels were all >4% in keeping with the findings of classical β-thalassaemia trait and significantly higher than levels seen in non-deletional forms of β-thalassaemia. The HbA2 level measured on the BioRad Variant II Hb analyser was lower than the level in the first description of the Filipino β-thalassaemia. β-thalassaemia trait with coinheritance of α-thalassaemia (-α) is associated with significantly higher HbA2 level.

Pharmacokinetics of Deferiprone in Patients with β-Thalassaemia : Impact of Splenectomy and Iron Status.

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Limenta, Lie Michael George; Jirasomprasert, Totsapol; Jittangprasert, Piyada; Wilairat, Prapin; Yamanont, Praveena; Chantharaksri, Udom; Fucharoen, Suthat; Morales, Noppawan Phumala

2011-01-01

Iron-rich transfusions and/or a compensatory increase in iron absorption ultimately result in iron loading in patients with β-thalassaemia. Hence, without iron chelation, iron accumulates relentlessly. Deferiprone has been shown to be capable of reducing the iron burden in patients with b-thalassaemia. However, there is wide interpatient variation in deferiprone-induced urinary iron excretion (UIE). We hypothesized that splenectomy and iron status might influence the pharmacokinetic profiles of deferiprone in patients with β-thalassaemia/haemoglobin E, and the present study was aimed at examining this hypothesis. Thirty-one patients with β-thalassaemia/haemoglobin E (20 splenecto-mized and 11 non-splenectomized patients) were enrolled in the study. After an overnight fast, the subjects received a single oral dose of deferiprone 25 mg/kg of body weight. Blood samples were collected pre-dosing and at 15, 30, 45, 60, 90, 120, 180, 240, 300, 360 and 480 minutes after dosing. Urine output was pooled and collected at 0-2, 2-4, 4-8, 8-12 and 12-24 hour intervals. Serum and urine concentrations of deferiprone and its metabolite deferiprone glucuronide were determined using a validated high-performance liquid chromatography method. Serum deferiprone-chelated iron and UIE were determined using a validated colourimetric method. No significant difference in the pharmacokinetic parameters of non-conjugated deferiprone was observed between splenectomized and non-splenectomized patients. However, the maximum serum concentration (C max ) and the area under the serum concentration-time curve (AUC) from time zero to infinity (AUC∞) values of deferiprone glucuronide were significantly lower (both p values of serum deferiprone-chelated iron, as well as UIE, were significantly higher (p values 7.1 µmol/L, 1645 mmol · min/L and 77.1 mmol, respectively) than in non-splenectomized patients (median values 3.1 µmol/L, 545 mmol · min/L and 12.5 µmol, respectively). Urinary

Distribution of serum lipids and lipoproteins in patients with beta thalassaemia major; an epidemiological study in young adults from Greece

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Barbetseas John

2004-03-01

Full Text Available Abstract Background Beta-thalassaemia major (b-TM has been defined as a combination of chronic hemolytic anemia, iron storage disease and myocarditis, and it has been associated with premature death especially due to heart failure. To the best of our knowledge the status of blood lipids in these patients has rarely been investigated. Thus, we assessed the levels of lipids and lipoproteins in a sample of cardiovascular disease free adult men and women with b-TM. Methods During 2003 we enrolled 192 consecutive patients with b-TM that visited our Institution for routine examinations. The Institution is considered the major reference center for b-TM in Greece. Of the 192 patients, 88 were men (25 ± 6 years old and 104 women (26 ± 6 years old. Fasting blood lipid levels were measured in all participants. Results Data analysis revealed that 4% of men and 2% of women had total serum cholesterol levels > 200 mg/dl, and 11% of men and 17% of women had triglyceride levels > 150 mg/dl. In addition, mean HDL cholesterol levels were 32 ± 11 mg/dl in men and 38 ± 10 mg/dl in women, lipoprotein-a levels were 8.3 ± 9 mg/dl in men and 8.8 ± 9 mg/dl in women, apolipoprotein-A1 levels were 111 ± 17 mg/dl in men and 123 ± 29 mg/dl in women, and apolipoprotein-B levels were 60 ± 20 mg/dl in men and 59 ± 14 mg/dl in women. Total-to-HDL cholesterol ratios were 3.7 ± 1.2 and 3.8 ± 1.5 in men and women, respectively. Conclusions The majority of the patients had blood lipid levels (by the exception of HDL-cholesterol within the normal range, and consequently the prevalence of lipid and lipoprotein abnormalities was much lower as compared to the general population of the same age. Interestingly, is that the total – to HDL cholesterol ratio was high in our patients, and may underline the importance of this index for the prognosis of future cardiac events in these patients.

Clinical efficacy and safety evaluation of tailoring iron chelation practice in thalassaemia patients from Asia-Pacific: a subanalysis of the EPIC study of deferasirox.

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Viprakasit, Vip; Ibrahim, Hishamshah; Ha, Shau-Yin; Ho, Phoebe Joy; Li, Chi-Kong; Chan, Lee-Lee; Chiu, Chang-Fang; Sutcharitchan, Pranee; Habr, Dany; Domokos, Gabor; Roubert, Bernard; Xue, Hong-Ling; Bowden, Donald K; Lin, Kai-Hsin

2011-03-01

Although thalassaemia is highly prevalent in the Asia-Pacific region, clinical data on efficacy and safety profiles of deferasirox in patients from this region are rather limited. Recently, data from the multicentre Evaluation of Patients' Iron Chelation with Exjade (EPIC) study in 1744 patients with different anaemias has provided an opportunity to analyse 1115 thalassaemia patients, of whom 444 patients were from five countries in the Asia-Pacific region (AP) for whom thalassaemia management and choice of iron chelators were similar. Compared to the rest of the world (ROW), baseline clinical data showed that the AP group appeared to be more loaded with iron (3745.0 vs. 2822.0 ng/ml) and had a higher proportion on deferoxamine monotherapy prior to the study (82.9 vs. 58.9%). Using a starting deferasirox dose based on transfusional iron intake and tailoring it to individual patient response, clinical efficacy based on serum ferritin reduction in AP and ROW thalassaemia patients was similar. Interestingly, the AP group developed a higher incidence of drug-related skin rash compared to ROW (18.0 vs. 7.2%), which may indicate different pharmacogenetic backgrounds in the two populations. Our analysis confirms that, with appropriate adjustment of dose, deferasirox can be clinically effective across different regions, with manageable side effects.

Complementary and alternative medicine use in children with thalassaemia.

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Efe, Emine; Işler, Ayşegül; Sarvan, Süreyya; Başer, Hayriye; Yeşilipek, Akif

2013-03-01

The aims of this study were to: (1) determine the types of complementary and alternative medicine use among children with thalassaemia as reported by parents and (2) describe sociodemographic and medical factors associated with the use of such treatments in families residing in southern Turkey. Thalassaemia is one of the most common human genetic diseases. Despite the therapeutic efforts, patients will encounter a variety of physical and psychological problems. Therefore, the use of complementary and alternative medicines among children thalassaemia is becoming increasingly popular. This is a descriptive study of complementary and alternative medicine. This study was conducted in the Hematology Outpatient Clinic at Akdeniz University Hospital and in the Thalassemia Centre at Ministry of Health Antalya Education and Research Hospital, Antalya, Turkey, between January 2010-December 2010. Parents of 97 paediatric patients, among 125 parents who applied to the haematology outpatient clinic and thalassaemia centre between these dates, agreed to take part in the study with whom contact could be made were included. Data were collected by using a questionnaire. The proportion of parents who reported using one or more of the complementary and alternative medicine methods was 82·5%. Of these parents, 61·8% were using prayer/spiritual practice, 47·4% were using nutritional supplements and 35·1% were using animal materials. It was determined that a significant portion of the parents using complementary and alternative medicine use it to treat their children's health problems, they were informed about complementary and alternative medicine by their paediatricians and family elders, and they have discussed the use of complementary and alternative medicine with healthcare professionals. To sustain medical treatment and prognosis of thalassaemia, it is important for nurses to consult with their patients and parents regarding the use and potential risks of some complementary

Characterisation and confirmation of rare beta-thalassaemia mutations in the Malay, Chinese and Indian ethnic groups in Malaysia.

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Tan, Jin Ai Mary Anne; Chin, Pui See; Wong, Yean Ching; Tan, Kim Lian; Chan, Lee Lee; George, Elizabeth

2006-10-01

In Malaysia, about 4.5% of the Malay and Chinese populations are heterozygous carriers of beta-thalassaemia. The initial identification of rare beta-globin gene mutations by genomic sequencing will allow the development of simpler and cost-effective PCR-based techniques to complement the existing amplification refractory mutation system (ARMS) and gap-PCR used for the identification of beta-thalassaemia mutations. DNA from 173 beta-thalassaemia carriers and five beta-thalassaemia major patients from the Malay, Chinese and Indian ethnic groups were first analysed by ARMS and gap-PCR. Ninety-five per cent (174/183) of the 183 beta-globin genes studied were characterised using these two techiques. The remaining nine uncharacterised beta-globin genes (4.9%) were analysed using genomic sequencing of a 904 bp amplified PCR product consisting of the promoter region, exon 1, intervening sequence (IVS) 1, exon 2 and the 5' IVS2 regions of the beta-globin gene. The rare beta-globin mutations detected in the Chinese patients were CD27/28 (+C) and CD43 (GAG-TAG), and -88 (C-T) in an Indian patient. Beta-globin mutations at CD16 (-C), IVS1-1 (G-A), IVS2-1 (G-A), -86 (C-G) and Haemoglobin South Florida (CD1, GTG-ATG) were confirmed in the Malay patients. The seven rare beta-globin mutations and a rare haemoglobin variant confirmed in this study have been described in other populations but have not been previously described in Malaysian beta-thalassemia patients.

Extramedullary haematopoiesis in Thalassaemia: results of radiotherapy: a report of three patients

International Nuclear Information System (INIS)

Pistevou-Gompaki, K.; Paraskevopoulos, P.; Kotsa, K.; Skaragas, G.; Repanta, E.

1996-01-01

Extramedullary haematopoiesis is sometimes encountered in serve anaemia. Rarely, it may cause neurological symptoms, leading to spinal cord or cauda equina compression. Three patients with thalassaemia intermedia, who developed neurological complications, are described. The diagnoses were based on the clinical findings, computed tomography and magnetic resonance imaging. Small doses of radiotherapy (10-20 Gy in 5-10 fractions) relieved symptoms in all of these patients. Our experience supports the role of radiation therapy as a treatment for this complication. (Author)

Bone age estimation and prediction of final height in patients with {beta}-thalassaemia major: a comparison between the two most common methods

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Christoforidis, Athanasios; Katzos, George; Athanassiou-Metaxa, Miranda [Aristotle University of Thessaloniki, 1st Paediatric Department, Thessaloniki (Greece); Badouraki, Maria [Ippokratio Hospital, Paediatric Radiology Department, Thessaloniki (Greece)

2007-12-15

Thalassaemic patients are in need of frequent assessment of bone age because of growth failure and pubertal disorders. To compare the ''rapid'' Greulich and Pyle (G and P) method with the third edition of the Tanner and Whitehouse (TW3) method for determining skeletal maturity and predicting final height in thalassaemic patients. A total of 191 radiographs from 58 patients (28 male, 30 female) were retrospectively evaluated by two investigators, one for each method. In 47 radiographs from 15 patients having attained their adult height, predicted final height was calculated according to each method. The mean bone ages determined by both the G and P and TW3 methods were lower than mean chronological age, although the differences were not statistically significant (10.04 {+-} 3.69 years and 9.98 {+-} 3.39 years vs. 10.78 {+-} 3.96 years, respectively). Both methods had a tendency to over-estimate final height. Overall, the TW3 method seemed to be more accurate than the G and P method (mean absolute error 3.21 {+-} 2.51 years vs. 3.99 {+-} 2.99 years, respectively, P=0.048). The same method should be used when serial assessments are performed, as both methods provide similarly reliable, although not equivalent, results. The TW3 height prediction method seemed to be more accurate in patients with {beta}-thalassaemia major than the G and P method, albeit with a large confidence interval. (orig.)

Bone age estimation and prediction of final height in patients with β-thalassaemia major: a comparison between the two most common methods

International Nuclear Information System (INIS)

Christoforidis, Athanasios; Katzos, George; Athanassiou-Metaxa, Miranda; Badouraki, Maria

2007-01-01

Thalassaemic patients are in need of frequent assessment of bone age because of growth failure and pubertal disorders. To compare the ''rapid'' Greulich and Pyle (G and P) method with the third edition of the Tanner and Whitehouse (TW3) method for determining skeletal maturity and predicting final height in thalassaemic patients. A total of 191 radiographs from 58 patients (28 male, 30 female) were retrospectively evaluated by two investigators, one for each method. In 47 radiographs from 15 patients having attained their adult height, predicted final height was calculated according to each method. The mean bone ages determined by both the G and P and TW3 methods were lower than mean chronological age, although the differences were not statistically significant (10.04 ± 3.69 years and 9.98 ± 3.39 years vs. 10.78 ± 3.96 years, respectively). Both methods had a tendency to over-estimate final height. Overall, the TW3 method seemed to be more accurate than the G and P method (mean absolute error 3.21 ± 2.51 years vs. 3.99 ± 2.99 years, respectively, P=0.048). The same method should be used when serial assessments are performed, as both methods provide similarly reliable, although not equivalent, results. The TW3 height prediction method seemed to be more accurate in patients with β-thalassaemia major than the G and P method, albeit with a large confidence interval. (orig.)

Transfusion-related acute lung injury (TRALI in two thalassaemia patients caused by the same multiparous blood donor

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George J Kontoghiorghes

2017-10-01

Full Text Available Two separate episodes of transfusion-related acute lung injury (TRALI in thalassaemia patients caused by red blood cell transfusions from the same multiparous blood donor are reported. Both cases had the same symptomatology and occurred 10-60 minutes of transfusion. The patients presented dyspnea, sweating, fatigue, dizziness, fever, and sense of losing consciousness. The chest x-ray showed a pulmonary oedema-like picture with both lungs filled with fluid. The patients were treated in the intensive therapy unit. They were weaned off the ventilator and discharged following hospitalization 7 and 9 days respectively. The TRALI syndrome was diagnosed to be associated with HLA-specific donor antibodies against mismatched HLA-antigens of the transfused patients. Haemovigilance improvements are essential for reducing the morbidity and mortality in transfused patients. Blood from multiparous donors should be tested for the presence of IgG HLA-Class I and –Class II antibodies before being transfused in thalassaemia and other chronically transfused patients.

Frequency of Gγ-globin promoter -158 (C>T) XmnI polymorphism in patients with homozygous/compound heterozygous beta thalassaemia.

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Ali, Nadir; Ayyub, Muhammad; Khan, Saleem Ahmed; Ahmed, Suhaib; Abbas, Kazim; Malik, Hamid Saeed; Tashfeen, Sunila

2015-03-01

Response to hydroxyurea therapy in homozygous or compound heterozygous beta thalassaemia (BT) has been reported as more favourable in the presence of XmnI polymorphism. The prevalence of XmnI polymorphism may vary with BT phenotypes and genotypes, and differs geographically in distribution. Prevalence of XmnI polymorphism is not known in northern Pakistan. To determine the frequency of Gγ-globin promoter -158 (C>T) XmnI polymorphism (XmnI polymorphism) in patients with homozygous or compound heterozygous beta thalassaemia. Polymerase chain reaction (PCR) for common beta thalassaemia mutations and Gγ-globin promoter -158 (C>T) XmnI polymorphism was performed on 107 blood samples of transfusion dependent beta thalassaemia (BT) patients in Pakistan. One hundred samples of unrelated BT traits and 94 samples of healthy subjects as controls were also analysed for BT mutations and XmnI polymorphism. Out of 301 DNA samples, XmnI polymorphism was detected in 71(24%); in normal controls, XmnI polymorphism was detected in 34/94 (36%) subjects; while in homozygous/compound heterozygous BT, it was detected in 14/107(13%) patients (Fisher's exact test, p=.0002). In heterozygous BT group, XmnI polymorphism was detected in 23/100 subjects (Fisher's exact test, p=.03 with normal controls, and p=.049 with homozygous/compound heterozygous BT). The most common BT genotype was Frame Shift (Fr) 8-9/Fr 8-9, and none of the patients with this genotype had XmnI polymorphism. The second most common genotype was IVSI-5/IVSI-5; 4/26 (15%). Cases with this genotype had XmnI polymorphism. XmnI polymorphism in homozygous/compound heterozygous BT group is 13%. The most common genotype associated with XmnI polymorphism was IVSI-5/IVSI-5. Copyright © 2015 King Faisal Specialist Hospital & Research Centre. Published by Elsevier B.V. All rights reserved.

A Java-based electronic healthcare record software for beta-thalassaemia.

Science.gov (United States)

Deftereos, S; Lambrinoudakis, C; Andriopoulos, P; Farmakis, D; Aessopos, A

2001-01-01

Beta-thalassaemia is a hereditary disease, the prevalence of which is high in persons of Mediterranean, African, and Southeast Asian ancestry. In Greece it constitutes an important public health problem. Beta-thalassaemia necessitates continuous and complicated health care procedures such as daily chelation; biweekly transfusions; and periodic cardiology, endocrinology, and hepatology evaluations. Typically, different care items are offered in different, often-distant, health care units, which leads to increased patient mobility. This is especially true in rural areas. Medical records of patients suffering from beta-thalassaemia are inevitably complex and grow in size very fast. They are currently paper-based, scattered over all units involved in the care process. This hinders communication of information between health care professionals and makes processing of the medical records difficult, thus impeding medical research. Our objective is to provide an electronic means for recording, communicating, and processing all data produced in the context of the care process of patients suffering from beta-thalassaemia. We have developed - and we present in this paper - Java-based Electronic Healthcare Record (EHCR) software, called JAnaemia. JAnaemia is a general-purpose EHCR application, which can be customized for use in all medical specialties. Customization for beta-thalassaemia has been performed in collaboration with 4 Greek hospitals. To be capable of coping with patient record diversity, JAnaemia has been based on the EHCR architecture proposed in the ENV 13606:1999 standard, published by the CEN/TC251 committee. Compliance with the CEN architecture also ensures that several additional requirements are fulfilled in relation to clinical comprehensiveness; to record sharing and communication; and to ethical, medico-legal, and computational issues. Special care has been taken to provide a user-friendly, form-based interface for data entry and processing. The

Thalassaemia screening among students in a secondary school in Ampang, Malaysia.

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Jameela, S; Sabirah, S O Sharifah; Babam, J; Phan, C L; Visalachy, P; Chang, K M; Salwana, M A; Zuraidah, A; Subramanian, Y; Rahimah, A

2011-12-01

Thalassaemia is a common disorder in Malaysia. It is estimated that 4.5% of the population are carriers for beta- or alpha- thalassaemias. We set out to screen Form 4 students aged between 15 and 16 years old in a national school, for thalassaemia in March 2008. Written consent was obtained from 310 students. The carrier rate for the common thalassaemia syndromes was 6.8% (2.9% for beta-thalassaemia, 2.6% for HbE and 1.3% for two-gene deletion for alpha-thalassaemia). Carriers for beta-thalassaemia and two-gene deletion for alpha-thalassaemia were more common in the Chinese (4.3% and 1.4% respectively) while heterozygous HbE was more common in the Malays (3.8%). The laboratory cost of screening one student was RM 45 and the total number of man-hours spent in this screening activity was 600. This screening exercise showed that thalassaemia carriers are common among the Chinese and Malays and it is feasible to carry out a screening programme for secondary school students.

Sickle cell disease in Madhya Pradesh, Central India: A comparison of clinical profile of sickle cell homozygote vs. sickle-beta thalassaemia individuals.

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Yadav, Rajiv; Lazarus, Monica; Ghanghoria, Pawan; Singh, Mpss; Gupta, Rasik Behari; Kumar, Surendra; Sharma, Ravendra K; Shanmugam, Rajasubramaniam

2016-10-01

The clinical manifestation in sickle cell disease (SCD) patients varies from one individual to another due to factors like the presence of alpha-thalassaemia mutation, foetal haemoglobin, and β-globin gene haplotype. The present study enumerates the clinical profile of sickle cell anaemia patients from Central India. Seven hundred seventy-six SCD patients from Jabalpur and surrounding districts (Madhya Pradesh) in central India were registered with the sickle cell clinic of NIRTH, Jabalpur. The present study reveals recorded signs and symptoms of genetically confirmed sickle cell anaemia (404) and sickle beta thalassaemia (92) patients. Majority of the patients were from scheduled caste communities (47.9%) and Gond tribal community (13.8%). Splenomegaly was the most common clinical manifestation observed (71.4%). Overall, 63.5% patients had a history of blood transfusion. The most frequent signs and symptoms observed were Pallor, Icterus, Joint pain, Fever, and Fatigue. Majority of the patients revealed onset of disease prior to attaining the age of 3 years (sickle cell anaemia 44.3% and sickle beta thalassaemia 35.9%). Mean haemoglobin levels among SCA individuals were marginally higher than SBT patients. On the other hand, mean foetal haemoglobin levels among SBT individuals showed the reverse trend. Notably, the present study reports the first incidence of priapism recorded in Central India. The study revealed a high prevalence of SCD among scheduled caste, backward caste, and tribal communities. Dissemination of study findings, screening, pre-marriage counselling, and pre-natal diagnosis are fundamental to preventing or lowering of birth of sickle cell anaemia children in the affected populations.

Prevalence of α-thalassaemia genotypes in pregnant women in northern Thailand

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Pharephan, Somphon; Sirivatanapa, Pannee; Makonkawkeyoon, Sanit; Tuntiwechapikul, Wirote; Makonkawkeyoon, Luksana

2016-01-01

Background & objectives: Alpha-thalassaemias are genetic disorders with high prevalence in northern Thailand. However, common genotypes and current data on the prevalence of α-thalassaemias have not been reported in this region. Therefore, the objective of the present study was to determine the prevalence of α-thalassaemia genotypes in pregnant women in northern Thailand. Methods: Genomic DNA was extracted from blood samples of pregnant women who came to Maharaj Nakorn Chiang Mai University Hospital during July 2009 to 2010. The common deletion and point mutation genotypes of α-thalassaemia were evaluated by gap- polymerase chain reaction (PCR) and PCR with restriction fragment length polymorphism (RFLP). Results: Genotypes of 638 pregnant women were: 409 samples (64.11%) being normal subjects (αα/αα) and 229 samples (35.89%) with α-thalassaemias. These 229 samples could be classified into deletional HbH disease (--SEA/-α3.7) for 18 samples (2.82%); heterozygous α0-thalassaemia --SEA type (--SEA/αα)) for 78 (12.23%); heterozygous α+-thalassaemia - α3.7 type (-α3.7/αα) for 99 (15.52%); homozygous α+-thalassaemia - α3.7 type (-α3.7/- α3.7) for five (0.78%); heterozygous α+-thalassaemia - α4.2 type (-α4.2/αα) for two (0.31%); and heterozygous HbCS (αCSα/αα) for 27 (4.23%) cases. Interpretation & conclusions: The prevalence of α-thalassaemias in pregnant women in northern Thailand was high. This finding supports the implementation of the prevention and control of this common genetic disorder by screening for α-thalassaemia genotypes. PMID:27241645

Comparing the Efficacy of Tadalafil Versus Placebo on Pulmonary Artery Systolic Pressure and Right Ventricular Function in Patients with Beta-Thalassaemia Intermedia.

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Jalalian, Rozita; Moghadamnia, Ali Akbar; Tamaddoni, Ahmad; Khafri, Soraya; Iranian, Mohammadreza

2017-07-01

Conventional oral therapies in the management of pulmonary hypertension in people without haemoglobinopathies are of limited value in thalassaemia patients because of toxicity and poor effectiveness. This study was conducted to assess the effect of tadalafil on pulmonary artery pressure and right ventricular systolic function in patients with beta-thalassaemia intermedia. Forty-four patients with beta-thalassaemia intermedia with pulmonary hypertension based on transthoracic echocardiography (TTE) were entered in the study. Patients with hepatic or renal insufficiency and also patients who were treated with organic nitrates or alpha-blockers were excluded. The patients were randomly divided into two groups (n=22) and they were treated for six weeks with tadalafil (40mg daily) or placebo. The pulmonary artery systolic pressure (PASP), tricuspid regurgitation velocity (TRV) and parameters related to systolic function of the right ventricle were measured by the TTE before and after treatment. Significant improvement in TRV (3.02±0.02 m/s-2.52±0.06 m/s), PASP (45.31±0.66 mmHg-34.26±1.15mmHg) and parameters related to systolic function of the right ventricle were observed in the group who received tadalafil compared to placebo (pintermedia. Likewise, tadalafil improved right ventricular systolic function in the patients. Copyright © 2016 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

Transfusion independence and HMGA2 activation after gene therapy of human β-thalassaemia.

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Cavazzana-Calvo, Marina; Payen, Emmanuel; Negre, Olivier; Wang, Gary; Hehir, Kathleen; Fusil, Floriane; Down, Julian; Denaro, Maria; Brady, Troy; Westerman, Karen; Cavallesco, Resy; Gillet-Legrand, Beatrix; Caccavelli, Laure; Sgarra, Riccardo; Maouche-Chrétien, Leila; Bernaudin, Françoise; Girot, Robert; Dorazio, Ronald; Mulder, Geert-Jan; Polack, Axel; Bank, Arthur; Soulier, Jean; Larghero, Jérôme; Kabbara, Nabil; Dalle, Bruno; Gourmel, Bernard; Socie, Gérard; Chrétien, Stany; Cartier, Nathalie; Aubourg, Patrick; Fischer, Alain; Cornetta, Kenneth; Galacteros, Frédéric; Beuzard, Yves; Gluckman, Eliane; Bushman, Frederick; Hacein-Bey-Abina, Salima; Leboulch, Philippe

2010-09-16

The β-haemoglobinopathies are the most prevalent inherited disorders worldwide. Gene therapy of β-thalassaemia is particularly challenging given the requirement for massive haemoglobin production in a lineage-specific manner and the lack of selective advantage for corrected haematopoietic stem cells. Compound β(E)/β(0)-thalassaemia is the most common form of severe thalassaemia in southeast Asian countries and their diasporas. The β(E)-globin allele bears a point mutation that causes alternative splicing. The abnormally spliced form is non-coding, whereas the correctly spliced messenger RNA expresses a mutated β(E)-globin with partial instability. When this is compounded with a non-functional β(0) allele, a profound decrease in β-globin synthesis results, and approximately half of β(E)/β(0)-thalassaemia patients are transfusion-dependent. The only available curative therapy is allogeneic haematopoietic stem cell transplantation, although most patients do not have a human-leukocyte-antigen-matched, geno-identical donor, and those who do still risk rejection or graft-versus-host disease. Here we show that, 33 months after lentiviral β-globin gene transfer, an adult patient with severe β(E)/β(0)-thalassaemia dependent on monthly transfusions since early childhood has become transfusion independent for the past 21 months. Blood haemoglobin is maintained between 9 and 10 g dl(-1), of which one-third contains vector-encoded β-globin. Most of the therapeutic benefit results from a dominant, myeloid-biased cell clone, in which the integrated vector causes transcriptional activation of HMGA2 in erythroid cells with further increased expression of a truncated HMGA2 mRNA insensitive to degradation by let-7 microRNAs. The clonal dominance that accompanies therapeutic efficacy may be coincidental and stochastic or result from a hitherto benign cell expansion caused by dysregulation of the HMGA2 gene in stem/progenitor cells.

Anabolic therapy in b-thalassaemia major induced osteoporosis: case report and literature review

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F.P. Cantatore

2011-06-01

Full Text Available Transfusion program and chelating therapy treatment has extended the life expectancy of thalassaemic patient; osteoporosis is considered an important cause of morbidity in adult patients who display increased fracture risk. This is a case report is about a thalassaemic young female with multiple spine fractures (D11, D12 e L2 and lumbar spine DEXA - Tscore = -3,1 and femoral = -3,4. This was in spite of therapy with alendronate 70 mg/week from January 2006 to September 2007. The patient was subsequentently treated for 18 months with 1-34 recombinant human parathyroid hormone and colecalciferol (100.000 U/monthly. After 4 months of therapy, the patient showed a decrease in spinal pain (Roland and Morris Disability Questionnaire and an improvement of quality of life (Qualeffo with normalization of osteocalcin and 25-OHcolecalciferol haematic levels after 6 months. Lumbar spine and femoral DEXA - Tscore, at 18 months, rose respectively to -2,5 and -2,4. Thalassaemia-induced osteoporosis is multifactorial and its management is very difficult. Bone marrow expansion, endocrine dysfunction, iron overload and genetic factors all seem to play important roles in the development of low bone mass in these patients. Bisphosfonates have been used in the management of thalassemia induced osteoporosis but there is no data about fracture risk. Anabolic therapy for thalassemic patients requests additional study on a large scale.

Detecting β-thalassaemia mutations from a single cell by PEP and RDB

Institute of Scientific and Technical Information of China (English)

YI Ping; LI Li; YAO Hong; ZHOU Yuan-guo; DENG Bing; CHEN Zhu-qin

2006-01-01

Objective:To evaluate the possibility of the technology involving PEP and RDB for detecting β-thalassaemia multipoint mutations from a single cell simultaneously. Methods: A set of allele specific oligonucleotide (ASO) probes used for detecting 8 familiar β-thalassaemia mutations (CD41-42, IVS- Ⅱ -654, CD17, TATA box nt-28, CD71-72, TATA box nt-29, CD26, IVS- Ⅰ -5) were immobilized on a strip of nylon membrane. The genome of a individual cell was amplified by primer extension preamplification (PEP) with the mixture of15-base random oligonucleotides. The aliquots from PEP were used to amplify the objective gene fractions of β-thalassaemia gene by nested or semi-nested PCR. The membrane was hybridized with the final amplified products and then treated with Streptavidin-HRP and color development.Results :Totally 30 lymphocytes were picked up from blood samples of 1 healthy female and 4 patients with known β-thalassaemia mutations respectively. Each single lymphocyte was lysed in the proteinase K buffer. The amplification efficacy was 94.0% and alle drop-out(ADO) rate was 8.0%. Revert dot blot (RDB) was applied to the final amplified products from the 5 participants. The results of diagnosis were the same to the expected, and their genotypes were N/N, CD17 (A→T)/N, IVS- Ⅱ -654(C→T)/CD17(A → T), CD41-42 (-CTTT)/N and TATA box nt-28 (A→G)/N, respectively. Conclusion: The technology involving PEP and RDB could detectmultiple β-thalassaemia mutations from a single cell simultaneously,and the research provides experimental evidences for the feasibility of applying PEP and DNA array technology to screening multiple genetic mutations from a single cell, and will be applied to preimplantation genetic diagnosis and non-invasive prenatal diagnosis for β-thalassaemia.

Epidural extramedullary haemopoiesis in thalassaemia

International Nuclear Information System (INIS)

Boyacigil, S.; Ali, A.; Ardic, S.; Yuksel, E.

2002-01-01

lntrathoracic extramedullary haematopoiesis is a rare condition. Involvement of the spinal epidural space with haematopoietic tissue is rather unusual. A 31-year-old-man with a known diagnosis of β-thalassaemia was referred with focal back pain. Magnetic resonance imaging revealed diffuse bone-marrow changes, thoracic paraspinal masses and lobulated epidural masses, suggesting extramedullary haemopoiesis. The patient was treated with radiotherapy and blood transfusions. Follow-up MRI was performed for evaluation efficacy of the treatment. Copyright (2002) Blackwell Science Pty Ltd

Hydroxyurea appears beneficial in patients with beta-thalassaemia major and intermedia

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S. F.S.A. Abdul Wahid

2007-06-01

Full Text Available Patients with severe inherited β-globin chain disorders may have milder illness if they produce high levels of fetal hemoglobin (HbF. Hydroxyurea (HU has been shown to enhance HbF levels in patients with sickle cell disease and may be useful in β-thalassemias. We administered HU to 13 patients with β-thalassemia intermedia or major, including 6 splenectomized patients. The patients received escalating doses (10 to 25 mg/kg/d of HU for around 2 years (median: 21 months, range: 8 - 55 months. Eleven patients responded with an increase in the pre-transfusion HbF levels, from a base line median of 8.0% (2.5 - 61.3% to 28.0% (6.6 - 49.2% and 40.7% (4.8 - 72.3% at 3 months and 18 months post-HU, respectively. A concomitant increment in median hemoglobin levels was noted at 1, 3 and 18 months of HU therapy. Six of 7 transfusion-dependent patients who had an increment of HbF (one with β-thalassemia major also had reduced transfusion requirement over the 2-year period of HU therapy. Response to HU was also shown by a reduction in spleen size. Apart from oral ulcers that resolved upon dose reduction of HU, no significant toxicity was noted. We conclude that increased HbF production in β-thalassemia patients, with an improvement in erythropoiesis, can be achieved using HU with minimal toxicity. (Med J Indones 2007; 16:78-83 Keywords: fetal hemoglobin (HbF, erythropoiesis, toxicity

Common Β- Thalassaemia Mutations in

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P Azarfam

2005-01-01

Full Text Available Introduction: β –Thalassaemia was first explained by Thomas Cooly as Cooly’s anaemia in 1925. The β- thalassaemias are hereditary autosomal disorders with decreased or absent β-globin chain synthesis. The most common genetic defects in β-thalassaemias are caused by point mutations, micro deletions or insertions within the β-globin gene. Material and Methods: In this research , 142 blood samples (64 from childrens hospital of Tabriz , 15 samples from Shahid Gazi hospital of Tabriz , 18 from Urumia and 45 samples from Aliasghar hospital of Ardebil were taken from thalassaemic patients (who were previously diagnosed .Then 117 non-familial samples were selected . The DNA of the lymphocytes of blood samples was extracted by boiling and Proteinase K- SDS procedure, and mutations were detected by ARMS-PCR methods. Results: From the results obtained, eleven most common mutations,most of which were Mediterranean mutations were detected as follows; IVS-I-110(G-A, IVS-I-1(G-A ،IVS-I-5(G-C ,Frameshift Codon 44 (-C,( codon5(-CT,IVS-1-6(T-C, IVS-I-25(-25bp del ,Frameshift 8.9 (+G ,IVS-II-1(G-A ,Codon 39(C-T, Codon 30(G-C the mutations of the samples were defined. The results showed that Frameshift 8.9 (+G, IVS-I-110 (G-A ,IVS-II-I(G-A, IVS-I-5(G-C, IVS-I-1(G-A , Frameshift Codon 44(-C , codon5(-CT , IVS-1-6(T-C , IVS-I-25(-25bp del with a frequency of 29.9%, 25.47%,17.83%, 7.00%, 6.36% , 6.63% , 3.8% , 2.5% , 0.63% represented the most common mutations in North - west Iran. No mutations in Codon 39(C-T and Codon 30(G-C were detected. Cunclusion: The frequency of the same mutations in patients from North - West of Iran seems to be different as compared to other regions like Turkey, Pakistan, Lebanon and Fars province of Iran. The pattern of mutations in this region is more or less the same as in the Mediterranean region, but different from South west Asia and East Asia.

Distribution of alpha thalassaemia in 16 year old Malaysian Students in Penang, Melaka and Sabah.

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Rahimah, A N; Nisha, S; Safiah, B; Roshida, H; Punithawathy, Y; Nurul, H; Syahzuwan, H; Zubaidah, Z

2012-12-01

Alpha thalassaemia is wide spread in Malaysia and is a public health problem. This study aimed to describe the carrier frequencies of α‒thalassaemia and its distribution among major ethnic groups in three states of Malaysia. Educational forums were organised and study was explained to students from three schools. Students were invited to take part in the screening with parent consent. A total of 8420 adolescent students aged 16 years volunteered to participate in the study. Peripheral blood samples were analysed for complete blood counts, haemoglobin quantification and typing, and serum ferritin levels. Genomic DNA was used for screening alpha thalassaemia alleles by PCR based molecular methods. We identified seven α‒globin gene defects in 341 (4.08%) students: amongst them α(+)‒ and α(0)‒thalassaemias were detected in 232 (2.77%) and 107 (1.28%) students respectively. Genotype ‒α(3.7)/αα was the most prevalent among sub-populations of Malay, indigenous communities of Sahab and Indian, while ‒‒(SEA)/αα deletion is more prevalent in Malaysian Chinese. It is estimated that 63 pregnancies annually are at risk of Hb Bart's hydrops fetalis. We have demonstrated the prevalence and mutation patterns of α‒thalassaemia in the 16 year olds in three states of Malaysia. High α(0)‒thalassaemia deletions amongst the study subjects place these carriers at an increased risk of conceiving fetuses with HbH disease and Hb Bart's hydrops fetalis should they choose another heterozygous partner. It is therefore highly recommended to institute community screening programmes and provide prospective carriers with genetic counselling to help them make informed choices.

α-Thalassaemia in Tunisia: some epidemiological and molecular data

Indian Academy of Sciences (India)

The aim of the present work is to acquire further data concerning -thalassaemia prevalence and molecular defects spectrum in Tunisia, by collecting and studying several kinds of samples carrying -thalassaemia. The first survey conducted on 529 cord blood samples using cellulose acetate electrophoresis, have ...

INSULIN-LIKE GROWTH FACTOR-1 (IGF-1: DEMOGRAPHIC, CLINICAL AND LABORATORY DATA IN 120 CONSECUTIVE ADULT PATIENTS WITH THALASSAEMIA MAJOR

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V. De Sanctis

2014-11-01

Full Text Available Introduction:  Insulin-like growth factor 1 (IGF-1 is a key peptide involved in cell growth and protein turnover, acting as the primary mediator of many of the responses regulated by growth hormone (GH in tissues. Signs and symptoms of adult GH deficiency (AGHD in patients with β-thalassaemia major (TM  may be subtle and overlap with those of the disease itself; therefore, the diagnosis may be missed or delayed, with potentially serious consequences. The diagnosis of AGHD requires an appropriate clinical setting and is confirmed through biochemical testing. The  aim of this study  was  to measure IGF-1 values and other clinical data in a large number of adult  patients with TM and to  evaluate whether an  IGF-1 concentration 2 SDs below normative values  could be used as an effective index supporting  the probable presence of  AGHD. Patients and Methods: A cohort of 120 adult patients with TM was studied for plasma levels of IGF-1. Plasma total IGF-1 was determined by chemiluminescent immunometric assay (CLIA method. In eleven patients (4 males the GH response during glucagon stimulation test (GST was also evaluated.  Results:  Fifty percent of patients (33 males and 27 females had IGF-1 levels   -2SDs. In multivariate regression analyses,  height, weight, BMI, serum ferritin, ALT, HCV serology and left ventricular ejection fraction (LVEF were not significantly related to IGF-1,  but a significant correlation was found in females between HCV-RNA positivity and IGF-1, ALT and serum ferritin (p= 0.043. AGHD was diagnosed in 6 (4 males out of 11 patients (54.5% who had glucagon stimulation tests and in 5 out of 8 (62.5% with IGF-1 <-2SD. The mean age of patients with GHD was 39.3 years (range: 25-49 years versus 35.8 years (range: 27-45 years in non-GHD patients. A positive correlation between GH peak after GST and IGF-1 level was found (r: 0.6409; p: < 0.05. Conclusions: On the basis of the present results and data from the

DNA studies are necessary for accurate patient diagnosis in compound heterozygosity for Hb Adana (HBA2:c.179>A) with deletional or nondeletional α-thalassaemia.

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Tan, Jin Ai Mary Anne; Kho, Siew Leng; Ngim, Chin Fang; Chua, Kek Heng; Goh, Ai Sim; Yeoh, Seoh Leng; George, Elizabeth

2016-06-08

Haemoglobin (Hb) Adana (HBA2:c.179>A) interacts with deletional and nondeletional α-thalassaemia mutations to produce HbH disorders with varying clinical manifestations from asymptomatic to severe anaemia with significant hepatosplenomegaly. Hb Adana carriers are generally asymptomatic and haemoglobin subtyping is unable to detect this highly unstable α-haemoglobin variant. This study identified 13 patients with compound heterozygosity for Hb Adana with either the 3.7 kb gene deletion (-α(3.7)), Hb Constant Spring (HbCS) (HBA2:c.427T>C) or Hb Paksé (HBA2:429A>T). Multiplex Amplification Refractory Mutation System was used for the detection of five deletional and six nondeletional α-thalassaemia mutations. Duplex-PCR was used to confirm Hb Paksé and HbCS. Results showed 84.6% of the Hb Adana patients were Malays. Using DNA studies, compound heterozygosity for Hb Adana and HbCS (α(codon 59)α/α(CS)α) was confirmed in 11 patients. A novel point in this investigation was that DNA studies confirmed Hb Paksé for the first time in a Malaysian patient (α(codon 59)α/α(Paksé)α) after nine years of being misdiagnosis with Hb Adana and HbCS (α(codon 59)α/α(CS)α). Thus, the reliance on haematology studies and Hb subtyping to detect Hb variants is inadequate in countries where thalassaemia is prevalent and caused by a wide spectrum of mutations.

[Curative Effects of Hydroxyurea on the Patients with β-thalassaemia Intermadia].

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Huang, Li; Yao, Hong-Xia

2016-06-01

To investigate the clinical features of β-thalassaemia intermediate (TI) patients and the curative effect and side reactions of hydroxyurea therapys. Twenty nine patients with TI were divided into hydroxyurea therapy group and no hydroxyurea therapy group; the curative effect and side reactions in 2 groups were compared; the situation of blood transfusion in the 2 groups was evaluated. In hydroxyurea therapy group, the hemoglobin level increased after treatment for 3 months; the reticulocyte percentage obviously decreased after treatment for 12 months; the serum ferritin had been maintained at a low level; while in no hydroxyurea therapy group, the levels of hemoglobin and reticulocytes were not significantly improved after treatment, the serum ferritin level gradually increased. In hydroxyurea therapy group, 12 cases were out of blood transfusion after treatment for 12 months, effective rate of treatment was 85.71%; while in no hydroxyurea therapy group, the blood transfusion dependency was not improved after treatment. No serious side reactions were found in all the hydroxyurea treated patients. The hydroxyurea shows a better curative effect on TI patients, no serious side reactions occur in all the patients treated with hydroxyurea, but the long-term curative effect and side reactions should be observed continuously.

Iron overload across the spectrum of non-transfusion-dependent thalassaemias: role of erythropoiesis, splenectomy and transfusions.

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Porter, John B; Cappellini, Maria Domenica; Kattamis, Antonis; Viprakasit, Vip; Musallam, Khaled M; Zhu, Zewen; Taher, Ali T

2017-01-01

Non-transfusion-dependent thalassaemias (NTDT) encompass a spectrum of anaemias rarely requiring blood transfusions. Increased iron absorption, driven by hepcidin suppression secondary to erythron expansion, initially causes intrahepatic iron overload. We examined iron metabolism biomarkers in 166 NTDT patients with β thalassaemia intermedia (n = 95), haemoglobin (Hb) E/β thalassaemia (n = 49) and Hb H syndromes (n = 22). Liver iron concentration (LIC), serum ferritin (SF), transferrin saturation (TfSat) and non-transferrin-bound iron (NTBI) were elevated and correlated across diagnostic subgroups. NTBI correlated with soluble transferrin receptor (sTfR), labile plasma iron (LPI) and nucleated red blood cells (NRBCs), with elevations generally confined to previously transfused patients. Splenectomised patients had higher NTBI, TfSat, NRBCs and SF relative to LIC, than non-splenectomised patients. LPI elevations were confined to patients with saturated transferrin. Erythron expansion biomarkers (sTfR, growth differentiation factor-15, NRBCs) correlated with each other and with iron overload biomarkers, particularly in Hb H patients. Plasma hepcidin was similar across subgroups, increased with >20 prior transfusions, and correlated inversely with TfSat, NTBI, LPI and NRBCs. Hepcidin/SF ratios were low, consistent with hepcidin suppression relative to iron overload. Increased NTBI and, by implication, risk of extra-hepatic iron distribution are more likely in previously transfused, splenectomised and iron-overloaded NTDT patients with TfSat >70%. © 2016 The Authors. British Journal of Haematology published by John Wiley & Sons Ltd.

A case of selective mutism in an 8-year-old girl with thalassaemia major after bone marrow transplantation.

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Plener, P L; Gatz, S A; Schuetz, C; Ludolph, A G; Kölch, M

2012-01-01

Selective mutism is rare with a prevalence below 1% in the general population, but a higher prevalence in populations at risk (children with speech retardation, migration). Evidence for treatment strategies is hardly available. This case report provides information on the treatment of selective mutism in an 8-year-old girl with preexisting thalassaemia major. As medications she received penicillin prophylaxis (500000 IE/d) and deferasirox (Exjade; 20-25mg/kg/d), an iron chelator. The preexisting somatic disease and treatment complicated the treatment, as there are no data about pharmacological combination therapy. Psychotherapy in day treatment, supported by the use of the SSRI fluoxetine (10 mg), led to a decrease in the selective mutism score from 33 to 12 points, GAF improved by 21 points. Mean levels of fluoxetine plus norfluoxetine were 287.8 ng/ml without significant level fluctuations. © Georg Thieme Verlag KG Stuttgart · New York.

Increased microerythrocyte count in homozygous alpha(+-thalassaemia contributes to protection against severe malarial anaemia.

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Freya J I Fowkes

2008-03-01

Full Text Available The heritable haemoglobinopathy alpha(+-thalassaemia is caused by the reduced synthesis of alpha-globin chains that form part of normal adult haemoglobin (Hb. Individuals homozygous for alpha(+-thalassaemia have microcytosis and an increased erythrocyte count. Alpha(+-thalassaemia homozygosity confers considerable protection against severe malaria, including severe malarial anaemia (SMA (Hb concentration 1.1 x 10(12/l as a result of the reduced mean cell Hb in homozygous alpha(+-thalassaemia. In addition, children homozygous for alpha(+-thalassaemia require a 10% greater reduction in erythrocyte count than children of normal genotype (p = 0.02 for Hb concentration to fall to 50 g/l, the cutoff for SMA. We estimated that the haematological profile in children homozygous for alpha(+-thalassaemia reduces the risk of SMA during acute malaria compared to children of normal genotype (relative risk 0.52; 95% confidence interval [CI] 0.24-1.12, p = 0.09.The increased erythrocyte count and microcytosis in children homozygous for alpha(+-thalassaemia may contribute substantially to their protection against SMA. A lower concentration of Hb per erythrocyte and a larger population of erythrocytes may be a biologically advantageous strategy against the significant reduction in erythrocyte count that occurs during acute infection with the malaria parasite Plasmodium falciparum. This haematological profile may reduce the risk of anaemia by other Plasmodium species, as well as other causes of anaemia. Other host polymorphisms that induce an increased erythrocyte count and microcytosis may confer a similar advantage.

Diagnostic significance of red cell indices in beta-thalassaemia trait

International Nuclear Information System (INIS)

Hussain, Z.; Malik, N.; Chughtai, A.S.

2005-01-01

The purpose of this study was to evaluate the formulae for the diagnosis of beta-thalassemia trait cases in settings where electrophoreses is not available. The study included 50 cases of beta-thalassaemia trait already diagnosed by Hb electrophoresis. CBC samples were analyzed on Sysmex K4500 and red cell indices were used to evaluate formulae for differentiating beta thalassaemia trait from iron deficiency anemia. The formula MCV/RBC and MCH/RBC identified 56% of the cases. Formula MCV - (5 x Hb)- RBC - 8.4 identified 54% of beta thalassemia trait cases. The formula MCV x MCH identified 92% of cases. RBC indices given by 100 electronic counters can be used to differentiate iron deficiency anemia from beta-thalassaemia trait at least provisionally in areas where Hb electrophoresis is not available. (author)

Prevalence of thalassaemia, iron-deficiency anaemia and glucose-6-phosphate dehydrogenase deficiency among Arab migrating nomad children, southern Islamic Republic of Iran.

Science.gov (United States)

Pasalar, M; Mehrabani, D; Afrasiabi, A; Mehravar, Z; Reyhani, I; Hamidi, R; Karimi, M

2014-12-17

This study investigated the prevalence of iron-deficiency anaemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency and β-thalassaemia trait among Arab migrating nomad children in southern Islamic Republic of Iran. Blood samples were analysed from 134 schoolchildren aged child had G6PD deficiency. A total of 9.7% of children had HbA2 ≥ 3.5 g/dL, indicating β-thalassaemia trait (10.8% in females and 7.8% in males). Mean serum iron, serum ferritin and total iron binding capacity were similar in males and females. Serum ferritin index was as accurate as Hb index in the diagnosis of iron-deficiency anaemia. A high prevalence of β-thalassaemia trait was the major potential risk factor in this population.

Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI

International Nuclear Information System (INIS)

Aydingoez, Ue.; Oto, A.; Cila, A.

1997-01-01

Spinal epidural extramedullary haematopoiesis is very rare in thalassaemia. A 27-year-old man with thalassaemia intermedia presented with symptoms and signs of spinal cord compression. MRI showed a thoracic spinal epidural mass, representing extramedullary haematopoietic tissue, compressing the spinal cord. Following radiotherapy, serial MRI revealed regression of the epidural mass and gradual resolution of spinal cord oedema. (orig.)

Improving transfusion practice in transfusion dependent thalassaemia patients

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Chathupa Wickremaarachchi

2017-10-01

Full Text Available The aim of this study was to improve current transfusion practice in transfusiondependent thalassaemia patients by determining whether safe transition from triplewashed red cells (TWRC to leucodepleted red cells (LDRC, increasing transfusion rates, reducing the use of frusemide and creating uniform practice across patients is possible. In patients receiving regular transfusions (50, triple-washed red blood cells were changed to LDRC, transfusion rates were increased to 5 mL/kg/h (in line with the Cooley’s Foundation guidelines to a maximum of 300 mL/h and frusemide was ceased. Medical review occurred at completion of the transfusion. Of the 20 patients on TWRC, 18 were transitioned to leucodepleted red cells (90%. Recurrent allergic reactions in 2 patients required re-institution of TWRC. 7 of the 8 patients on regular frusemide ceased this practice with no documented transfusion-related fluid overload. One patient refused. Of the eligible 50 patients, 20 patients (40% were increased to the maximum transfusion rate of 300 mLs/h; 6 (12% increased rate but refused to go to the maximum; 9 (18% refused a change in practice and 15 (30% were already at the maximum rate. There was only one documented transfusion reaction (palpitations however this patient was able to tolerate a higher transfusion rate on subsequent transfusions. Thalassemia patients on TWRC were safely transitioned to LDRC. Transfusion rates were safely increased, with a calculated reduction in day-stay bed time of 17.45 h per month. This confirms a guideline of 5 mL/kg/h for transfusion-dependant thalassaemia patients with preserved cardiac function is well tolerated and may be translated to other centres worldwide.   本研究的目的是通过确定是否有可能进行从三洗红细胞（TWRC）到去白细胞红细胞（LDRC）的安全过渡，提高输血速率，减少速尿的使用，并在患者中实施统一规则，从而改进输血依赖型地中海贫血�

Diagnostic applications of newborn screening for α-thalassaemias, haemoglobins E and H disorders using isoelectric focusing on dry blood spots.

Science.gov (United States)

Jindatanmanusan, Punyanuch; Riolueang, Suchada; Glomglao, Waraporn; Sukontharangsri, Yaowapa; Chamnanvanakij, Sangkae; Torcharus, Kitti; Viprakasit, Vip

2014-03-01

Neonatal screening for haemoglobin (Hb) disorders is a standard of care in several developed countries with the main objective to detect Hb S. Such practice has not been established in Thailand where α-thalassaemia and haemoglobin E (Hb E) are highly prevalent. Early identification of thalassaemias could be helpful and strengthen the programme for prevention and control for severe thalassaemias. Data from isoelectric focusing (IEF) and Isoscan® for detecting types and amount (%) of each haemoglobin in 350 newborn's dried blood spots were analysed and compared with the comprehensive genotype analysis by DNA studies as a gold standard. Based on genetic profiles, there were 10 different categories: (1) normal (n = 227), (2) α(+)-thalassaemia trait (n = 14), (3) α(0)-thalassaemia trait (n = 13), (4) β(0)-thalassaemia trait (n = 7), (5) Hb E trait (n = 72), (6) Hb E trait with α(0)-thalassaemia or homozygous α(+)-thalassaemia (n = 5), (7) Hb E trait with α(+)-thalassaemia trait (n = 5), (8) homozygous Hb E (n = 3), (9) homozygous Hb E with α(0)-thalassaemia trait (n = 1) and (10) Hb H disease (n = 3). The presence of Hb Bart's and Hb E were used to identify cases with α-thalassaemia and Hb E, respectively. We set 0.25% of Hb Bart's and 1.5% of Hb E as a cut-off level to detect α(+)-thalassaemia trait (sensitivity 92.86% and specificity 74.0%) and Hb E trait with 100% of both sensitivity and specificity for IEF diagnosis. Although molecular diagnosis seems to be better for definitive diagnosis of thalassaemia syndromes at birth, however, using our reference range described herein, IEF can be applied in a resource-limiting setting with acceptable reliability.

THALASSAEMIA IS A TROPICAL DISEASE REVIEW ARTICLE

African Journals Online (AJOL)

sickle cell disease, thalassaemia and Glucose-6-. Phosphate .... abnormalities of the membrane cause premature destruction ... endocrine complications which includes failure of puberty ... Pulmonary hypertension is also a common cause of.

Evaluation of high performance liquid chromatography (HPLC) pattern and prevalence of beta-thalassaemia trait among sickle cell disease patients in Lagos, Nigeria.

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Adeyemo, Titilope; Ojewunmi, Oyesola; Oyetunji, Ajoke

2014-01-01

Sickle cell disease (SCD) is the most common inherited disorder of haemoglobin worldwide. This study evaluated the chromatographic patterns and red blood cell indices of sickle cell patients to determine the co-inheritance of other haemoglobin(Hb) variants and β-thalassaemia trait. Red cell indices, blood film, sickle solubility test, Hb electrophoresis using alkaline cellulose acetate membrane, and chromatographic patterns using Bio Rad HPLC Variant II were evaluated for 180 subjects. Based on low MCV 4.0% on HPLC and Hb variants eluting outside the S and C windows, at least four haemoglobin phenotypes (SS: 87.7%; SC: 1.1%; SD Punjab: 0.6%; Sβ-thalassemia: 10.6%) were identified. Mean Hb F% was 8.1±5.1 (median 7.65) for Hb SS and 6.03±5.2 (median 3.9) for Hb Sβ-thalassemia trait. Majority of Hb SS (69.1%) had Hb F% less than 10 while 27.6% had 10-19.9 and 3.2% had ≥ 20. Mean Hb F% was higher in female Hb SS (9.55±5.09; mean age 7.4±3.8 years) than the males (7.63±4.80; mean age 6.9±3.8 years) (P=0.02). A borderline significant negative correlation between age and Hb F levels among Hb SS subjects (r= -0.169 P=0.038) was also observed. Our data suggests that α and β- thalassaemia traits, and other haemoglobin variants co-exist frequently with SCD in our population.

Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI

Energy Technology Data Exchange (ETDEWEB)

Aydingoez, Ue.; Oto, A.; Cila, A. [Department of Radiology, Hacettepe University School of Medicine, Ankara (Turkey)

1997-12-01

Spinal epidural extramedullary haematopoiesis is very rare in thalassaemia. A 27-year-old man with thalassaemia intermedia presented with symptoms and signs of spinal cord compression. MRI showed a thoracic spinal epidural mass, representing extramedullary haematopoietic tissue, compressing the spinal cord. Following radiotherapy, serial MRI revealed regression of the epidural mass and gradual resolution of spinal cord oedema. (orig.) With 3 figs., 6 refs.

Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord

International Nuclear Information System (INIS)

Niggemann, P.; Krings, T.; Thron, A.; Hans, F.

2005-01-01

A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained

Fifteen-year follow-up of a patient with beta thalassaemia and extramedullary haematopoietic tissue compressing the spinal cord

Energy Technology Data Exchange (ETDEWEB)

Niggemann, P.; Krings, T.; Thron, A. [Dept. of Neuroradiology, RWTH-Aachen Hosital (Germany); Hans, F. [Dept. of Neurosurgery, RWTH Aachen Hospital (Germany); 1

2005-04-01

A long-term follow-up of a patient with beta thalassaemia with intra- and extraspinal extramedullary haematopoietic tissue compressing the spinal cord is presented. Extramedullary haematopoietic nodules are a rare cause of spinal cord compression and should be included in the differential diagnosis, especially in patients from Mediterranean countries. Treatment with radiation therapy solely failed, giving rise to the need of surgical intervention. Surgical decompression of the spine and the removal of the culprit lesion compressing the spine were performed. Postinterventional radiation therapy was applied to the spine. A relapse had to be treated again by surgical means combined with postinterventional radiation therapy. A complete relief of the symptoms and control of the lesion could be obtained.

Pre-marriage prevention of thalassaemia: report of a 100,000 case experience in Isfahan.

Science.gov (United States)

Ghanei, M; Adibi, P; Movahedi, M; Khami, M A; Ghasemi, R L; Azarm, T; Zolfaghari, B; Jamshidi, H R; Sadri, R

1997-05-01

Iran like other middle east countries has a large number of major thalassaemics. Due to religious restrictions on abortion, the routine prevention of the birth of thalassaemic children by this means is not possible. The aim of this study is to describe an alternative means to prevent the birth of thalassaemic children. From January 1993 to January 1996, 10,000 people preparing for marriage were screened for the thalassaemia trait, using CBC and HbA2 level measurement. High risk couples were referred for further consultation regarding the disease and the means of its prevention. The proposed actions of the couples regarding thalassaemia prevention were evaluated immediately after consultation and then re-evaluated three months later. After the project had been running for three years the average of high risk couple initially deciding not to marry was 90% and no new cases of thalassemia were detected in the children of the screened population. Where both members of the couple were trait-positive their preferred choice was not to marry, rather than to marry and use other or no methods of preventing a thalassemia affected child being born to them. Cultural and religious ideas can affect such decisions and in some Islamic countries the establishment and use of a genetic counselling centre can help prevent most of new thalassaemia cases.

Selected highlights of the VIII International Symposium of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) on Growth, Puberty and Endocrine Complications in Thalassaemia. Auditorium of the Sultan Qaboos University (SQU) Muscat (Sultanate of Oman), 20th of December 2014.

Science.gov (United States)

De Sanctis, Vincenzo; Soliman, Ashraf T; Wali, Yasser; Elsedfy, Heba; Daar, Shahina; Al-Yaarubi, Saif A H; Mevada, Surekha Tony; Tony, Surekha; Elshinawy, Mohamed; Fawzy, Hanan; Al-Subhi, Taimoora; Al-Rawas, Abulhakim; Al-Muslehi, Muhanna; El Kholy, Mohamed

2015-03-01

The VIII ICET-A International Symposium was held in Muscat (Sultanate of Oman) on the 20th of December, 2014. The symposium included four sessions on a wide range of topics covering growth disorders and endocrine complications in thalassaemia. Despite the fact that endocrine complications are very common in multi-transfused thalassaemia patients a recent survey conducted by the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescent Medicine (ICET-A) in 2014 in Acitrezza (Catania, Italy) showed that the major difficulties reported by hematologists or pediatricians experienced in thalassaemias or thalassaemia syndromes in following endocrine complications included: Lack of familiarity with medical treatment of endocrine complications, interpretation of endocrine tests, lack of collaboration and on-time consultation between thalassaemic centres supervised by haematologists and endocrinologists. Endocrine monitoring of growth, pubertal development, reproductive ability and endocrine function in general are essential to achieve a good quality of life as well as controlling the pain which results from the defects of bone structure, all of which increase with the age of patients. Such comprehensive care is best provided by coordinated, multidisciplinary teams working in expert centres. The multidisciplinary team must include an endocrinologist, preferably someone experienced in the management of hormonal deficiencies caused early in life by transfusion-induced iron overload.

Hydroxyurea for reducing blood transfusion in non-transfusion dependent beta thalassaemias.

Science.gov (United States)

Foong, Wai Cheng; Ho, Jacqueline J; Loh, C Khai; Viprakasit, Vip

2016-10-18

Non-transfusion dependent beta thalassaemia is a subset of inherited haemoglobin disorders characterised by reduced production of the beta globin chain of the haemoglobin molecule leading to anaemia of varying severity. Although blood transfusion is not a necessity for survival, it is required when episodes of chronic anaemia occur. This chronic anaemia can impair growth and affect quality of life. People with non-transfusion dependent beta thalassaemia suffer from iron overload due to their body's increased capability of absorbing iron from food sources. Iron overload becomes more pronounced in those requiring blood transfusion. People with a higher foetal haemoglobin level have been found to require fewer blood transfusions. Hydroxyurea has been used to increase foetal haemoglobin level; however, its efficacy in reducing transfusion, chronic anaemia complications and its safety need to be established. To assess the effectiveness, safety and appropriate dose regimen of hydroxyurea in people with non-transfusion dependent beta thalassaemia (haemoglobin E combined with beta thalassaemia and beta thalassaemia intermedia). We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled from electronic database searches and handsearching of relevant journals. We also searched ongoing trials registries and the reference lists of relevant articles and reviews.Date of last search: 30 April 2016. Randomised or quasi-randomised controlled trials of hydroxyurea in people with non-transfusion dependent beta thalassaemia comparing hydroxyurea with placebo or standard treatment or comparing different doses of hydroxyurea. Two authors independently applied the inclusion criteria in order to select trials for inclusion. Both authors assessed the risk of bias of trials and extracted the data. A third author verified these assessments. No trials comparing hydroxyurea with placebo or standard care were found. However, we included

CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT

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Athanasios Aessopos

2009-07-01

Full Text Available Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia.  The two main factors that determine cardiac disease in homozygous β thalassemia are the high output state that results from chronic tissue hypoxia, hypoxia-induced compensatory reactions and iron overload.  The high output state playing a major role in thalassaemia intermedia and the iron load being more significant in the major form. Arrhythmias, vascular involvement that leads to an increased pulmonary vascular resistance and an increased systemic vascular stiffness and valvular abnormalities also contribute to the cardiac dysfunction in varying degrees according to the severity of the phenotype.  Endocrine abnormalities, infections, renal function and medications can also play a role in the overall cardiac function.  For thalassaemia major, regular and adequate blood transfusions and iron chelation therapy are the mainstays of management. The approach to thalassaemia intermedia, today, is aimed at monitoring for complications and initiating, timely, regular transfusions and/or iron chelation therapy.  Once the patients are on transfusions, then they should be managed in the same way as the thalassaemia major patients.  If cardiac manifestations of dysfunction are present in either form of thalassaemia, high pre transfusion Hb levels need to be maintained in order to reduce cardiac output and appropriate intensive chelation therapy needs to be instituted.  In general recommendations on chelation, today, are usually made according to the Cardiac Magnetic Resonance findings, if available.  With the advances in the latter technology and the ability to tailor chelation therapy according to the MRI findings as well as the availability of three iron chelators, together with

Fertility and pregnancy in thalassaemia and sickle cell disease. The UK guidelines

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Bernard Davis

2014-12-01

Full Text Available Progressive improvements in the health and survival of patients with thalassaemia and sickle cell disease have increased the reproductive prospects of affected individuals. However, pregnancy in these disorders is associated with significant maternal and fetal risks and expert management is required to ensure good outcomes. In the United Kingdom, it is recognised that the patchy geographical distribution of these conditions poses challenges for access to specialist care, including specialist obstetric services. Guidelines on the pregnancy management of thalassaemia and sickle cell disease in the UK have been published by the Royal College of Obstetricians and Gynaecologists. These guidelines describe the preconceptual, antenatal, intrapartum and postpartum aspects of care. They highlight the high-risk status of pregnancy in these conditions and emphasise the vital importance of specialist multidisciplinary care to the achievement of favourable maternal and fetal outcomes. The guidelines are a valuable resource to healthcare professionals, especially those working in low prevalence areas.

A new tool for the assessment of satisfaction with iron chelation therapy (ICT-Sat) for patients with β-thalassemia major.

Science.gov (United States)

Elalfy, Mohsen S; Massoud, Walid; Elsherif, Nayera H; Labib, Jonair H; Elalfy, Omar M; Elaasar, Safaa; von Mackensen, Sylvia

2012-06-01

High satisfaction with iron chelation is a major determinant for adherence to ICT in beta-thalassaemia major (β-TM) patients. In this study, a new tool to assess different domains of satisfaction for available forms of ICT was developed and validated. The impact of patients' satisfaction with ICT has been tested. Items were generated via focus groups and a preliminary version with 24 items (ICT-Sat) with an additional item for treatment preference and a knowledge questionnaire (KQ) was developed. 170 β-TM patients from three Thalassaemia centers in Egypt, aged 2-32 years received three questionnaires to fill in; the new ICT-Sat, a KQ, and a previously validated tool for satisfaction with ICT (SICT) and retested 4-6 weeks later to ensure re-test reliability. Type of chelation, drug related adverse events, compliance with ICT, and serum ferritin level (SF) during the year prior to the study as well as available cardiac T2*data were recorded. One hundred and fifty two β-TM patients completed all questionnaires; median age was 12 years. The final 15 remaining ICT-Sat items, yielding to four domain scores, explained 70.6% of the total variance. The "perceived effectiveness" and "fear and worries" domains of the ICT-Sat correlated significantly with the domains "perceived effectiveness" and "acceptance" of the SICT. Patients treated with oral ICT were more satisfied with perceived effectiveness, and their side effects. A new clinically based ICT-Sat tool was developed and revealed good psychometric characteristics. Adherence to ICT was better correlated with "perceived effectiveness" and SF level. Copyright © 2012 Wiley Periodicals, Inc.

Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease.

Science.gov (United States)

Hussein, Norita; Weng, Stephen F; Kai, Joe; Kleijnen, Jos; Qureshi, Nadeem

2018-03-14

Globally, about five per cent of children are born with congenital or genetic disorders. The most common autosomal recessive conditions are thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease, with higher carrier rates in specific patient populations. Identifying and counselling couples at genetic risk of the conditions before pregnancy enables them to make fully informed reproductive decisions, with some of these choices not being available if genetic counselling is only offered in an antenatal setting. This is an update of a previously published review. To assess the effectiveness of systematic preconception genetic risk assessment to improve reproductive outcomes in women and their partners who are identified as carriers of thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease in healthcare settings when compared to usual care. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Registers. In addition, we searched for all relevant trials from 1970 (or the date at which the database was first available if after 1970) to date using electronic databases (MEDLINE, Embase, CINAHL, PsycINFO), clinical trial databases (National Institutes of Health, Clinical Trials Search portal of the World Health Organization, metaRegister of controlled clinical trials), and hand searching of key journals and conference abstract books from 1998 to date (European Journal of Human Genetics, Genetics in Medicine, Journal of Community Genetics). We also searched the reference lists of relevant articles, reviews and guidelines and also contacted subject experts in the field to request any unpublished or other published trials.Date of latest search of the registers: 20 June 2017.Date of latest search of all other sources: 16 November 2017. Any randomised or quasi-randomised controlled trials (published or unpublished) comparing reproductive outcomes of systematic preconception genetic risk assessment for thalassaemia, sickle

Choosing offspring: prenatal genetic testing for thalassaemia and the production of a ‘saviour sibling’ in China

NARCIS (Netherlands)

Sui, S.

2010-01-01

This paper focuses on the pre-natal genetic testing and reproductive decision-making around thalassaemia in China. Findings are based on fieldwork conducted in hospitals and research institutions, interviews with families with thalassaemia-affected children, interviews with geneticists and genetic

Red blood-cell alloantibodies in multiply transfused patients in the occupied Palestinian territory: a pilot study.

Science.gov (United States)

Yaseen, Ahmad; Suleiman, Sa'd; Zenah, Omar Abu; Abu Taha, Adham

2018-02-21

Red blood-cell transfusion has greatly reduced the mortality and morbidity in multiply transfused patients with thalassaemia and sickle cell disease. However, this can result in red blood-cell isoimmunisation with autoantibodies and alloantibodies, which can lead to serious complications such as delayed haemolytic transfusion reaction. The aim of this study was to assess the frequency and types of alloantibodies in multiply transfused patients living in the north of the West Bank. This pilot study was done at three thalassaemia centres in Nablus, Jenin, and Tulkarm in the occupied Palestinian territory where 300 patients with thalassaemia and sickle cell anaemia regularly receive blood transfusions. Alloantibody screening and identification were done using three-cell and eleven-cell panels (DiaPanel, Bio-rad, Switzerland) respectively. Ethical approval was obtained from Institutional Review Board Centre at Najah University. Written consent was obtained from participants. 131 patients were enrolled. Of the 20 (15%) patients with alloantibodies, 14 (70%) were diagnosed with β-thalassaemia major, three (15%) were diagnosed with sickle cell anaemia, two (10%) were diagnosed with thalassaemia intermedia, and one (5%) was diagnosed with sickle cell thalassaemia. 13 (65%) patients had alloantibodies that belonged to the Rh blood group system (nine [45%] patients had anti-D; two [10%] had anti-E; one [5%] had anti Rh-C; and one [5%] had anti-c). Anti-Kell was found in seven (35%) patients. Our data showed a quite high prevalence of alloimmunisation in multiply transfused patients. Rh and Kell blood group system antibodies were the only alloantibodies identified in this study. To reduce alloimmunisation, it will be essential to introduce a policy for extended red blood-cell phenotyping of these patients and for the issuing of antigen-matched blood (at least for Rh and Kell antigen). Najah National University. Copyright © 2018 Elsevier Ltd. All rights reserved.

VITAMIN D STATUS IN CHILDREN WITH THALASSAEMIA IN NORTH EAST INDIA

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Dipangkar Hazarika

2016-11-01

Full Text Available BACKGROUND The aim of the study was to determine the frequency of vitamin D deficiency and insufficiency in children with thalassaemia who were on regular blood transfusion. MATERIALS AND METHODS Total 25 children were included in this retrospective study in the age group of 2-14 years with confirmed diagnosis of thalassaemia syndrome. They were on regular blood transfusion every 3-4 weeks and they had serum ferritin >1000 µg/L irrespective of chelation therapy. Serum level of calcium, phosphorous, alkaline phosphatase, serum ferritin and 25OH vitamin D were estimated. RESULTS Out of 25 patients, 6 (24% showed sufficient amount of vitamin D, 6 (24% showed insufficiency, 10 (40% showed deficiency and 3 (12% showed severe deficiency. Children with vitamin D deficiency showed high level of serum ferritin level. Severe vitamin D deficiency was seen when number of transfusions were >20.14 children showed abnormal vitamin D level when their serum ferritin level was between 1000-2000 ng/dL. There was no association between use of iron-chelating agent, duration of their use and nutritional status with vitamin D level. CONCLUSION In regularly transfused thalassaemic children, vitamin D insufficiency and deficiency were common and they need frequent monitoring for early detection and management.

Prenatal diagnosis of beta-thalassaemia: experience in a developing country.

Science.gov (United States)

Saxena, R; Jain, P K; Thomas, E; Verma, I C

1998-01-01

We present our experience with the amplification refractory mutation system (ARMS) for the prenatal diagnosis of beta-thalassaemia in 415 pregnancies of 360 women. Five mutations of the beta-thalassaemia gene common in Asian Indians accounted for 89.2 per cent and rare mutations for 7.2 per cent of all mutant chromosomes, while 3.3 per cent of chromosomes remained uncharacterized. Identical mutations were present in both parents in 43.2 per cent of cases, due to caste-based marriages in India. A confirmed diagnosis was given in 401 (98.3 per cent) cases, of which a complete diagnosis (whether the fetus was normal, a carrier, or homozygous) was possible in 391 (94.2 per cent) of the cases. In 15 couples, the mutation was identified in only one parent. In nine of these, the identified mutation was not present in the fetus, predicting normal/carrier status, while in five the identified mutation was present in the fetus, suggesting carrier/affected status. The abortion rate was 3.9 per cent. Pitfalls in diagnosis were failure of oligonucleotides to work, maternal contamination, and false paternity. The ARMS provides an inexpensive, robust and non-isotopic method for the prenatal diagnosis of beta-thalassaemia in India. Recommendations are outlined for establishing a prenatal diagnostic service in developing countries.

Alpha thalassemia among sickle cell anaemia patients in Kampala, Uganda.

Science.gov (United States)

Lubega, Irene; Ndugwa, Christopher M; Mworozi, Edison A; Tumwine, James K

2015-06-01

Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described. To determine the prevalence of α+thalassaemia among sickle cell anaemia patients in Mulago Hospital and to describe the clinical and laboratory findings in these patients. A cross sectional study was carried out on patients with sickle cell anaemia in Kampala. Dried blood spots were used to analyze for the deletional α+ thalassaemia using multiplex polymerase chain reaction. Of the 142 patients with sickle cell anaemia, 110 (77.5%) had the αα+thalassaemia deletion. The gene frequency of (-α) was 0.425. Ninety one percent (100/110) of those with α+thalassaemia were heterozygous (αα/α-). Amongst the patients older than 60 months, 15 (83.3%) of those without αα+thalassaemia had significant hepatomegaly of greater than 4 cm compared to 36 (45.6%) of those with α+thalassaemia (p=0.003). The gene frequency of (-α) of 0.425 noted in this study is higher than that reported from many places in Africa. Concurrent alpha thalassemia might be a protective trait against significant hepatomegaly in sickle cell anaemia patients more than 60 months of age at Mulago hospital.

Effects of iron salts and haemosiderin from a thalassaemia patient on oxygen radical damage as measured in the comet assay

NARCIS (Netherlands)

Anderson, D.; Yardley-Jones, A.; Hambly, R.J.; Vives-Bauza, C.; Smykatz-Kloss, V.; Chua-anusorn, W.; Webb, J.

2000-01-01

Thalassaemia is a group of genetic diseases where haemoglobin synthesis is impaired. This chronic anaemia leads to increased dietary iron absorption, which develops into iron overload pathology. Treatment through regular transfusions increases oxygen capacity but also provides iron through the red

beta-thalassaemia major hos børn og unge i Danmark

DEFF Research Database (Denmark)

Jung, Anne; Main, Katharina Maria; Scheibel, Elma

2002-01-01

INTRODUCTION: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment...... strategy. MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters...... were performed. RESULTS: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin

Beta-thalassaemia major hos børn og unge i Danmark

DEFF Research Database (Denmark)

Jung, A.; Main, K.M.; Scheibel, E.

2002-01-01

INTRODUCTION: Beta-thalassemia major occurs with increasing frequency among Danish children as a result of immigration. The aim of the study was to estimate the occurrence of beta-thalassemia major in Denmark, analyse the treatment and organ functions, and identify areas for an improved treatment...... strategy. MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters...... were performed. RESULTS: Twenty-six children had beta-thalassemia major. Out of these, 20 received blood transfusions, and 17 patients were chelated. Eight patients were not chelated owing to previous bone marrow transplantation, treatment with hydroxyurea or ferritin

Choosing offspring: prenatal genetic testing for thalassaemia and the production of a 'saviour sibling' in China.

Science.gov (United States)

Sui, Suli; Sleeboom-Faulkner, Margaret

2010-02-01

This paper focuses on the pre-natal genetic testing and reproductive decision-making around thalassaemia in China. Findings are based on fieldwork conducted in hospitals and research institutions, interviews with families with thalassaemia-affected children, interviews with geneticists and genetic researchers and a literature review conducted between September and November 2007. The paper aims to provide insight into the ways in which those who carry thalassaemia decide to have a test for the condition and the choices available to prospective parents. The paper also analyses factors affecting reproductive choices and the decision to produce a 'saviour sibling', including financial implications, state family planning policy, images and information conveyed through the media and propaganda, advice and counselling from doctors, psychological pressure from the community and social discrimination. The paper concludes with a discussion on the issues involved in the creation of saviour siblings, some of which are particular to China.

Role of genetic counselling in prenatal diagnosis of beta-thalassaemia in pakistan

International Nuclear Information System (INIS)

Bozdar, M.; Hanif, T.B.

2013-01-01

To compare the response towards prenatal diagnosis (PND) of b-thalassaemia, in individuals who had not received genetic counselling and a genetically counselled population. Study Design: Cross-sectional survey. Place and Duration of Study: Department of Haematology, Armed Forces Institute of Pathology (AFIP), Rawalpindi, from March 2009 to December 2010. Methodology: Using non-probability consecutive sampling, a total of 176 individuals having thalassaemic children, were interviewed regarding PND of thalassaemia, by using a structured questionnaire. Forty two individuals were taken as controls as they had received genetic counselling for PND, whereas the remaining 134 were taken as cases. Responses towards PND were compared using chi-square test. Odds ratio was also calculated for subsequent PND utilization. Results: Seventy (52.2%) cases and 42 (100%) controls were aware of the availability of PND in Pakistan. This difference in awareness was statistically significant (p < 0.001). In the controls, 40 (95.3%) individuals were aware of the appropriate timing of the test, in contrast to 52 (39%) cases (p < 0.001). PND was used in subsequent pregnancies by 50 (37.3%) cases and 32 (80%) controls (p < 0.001). The calculated odds ratio for subsequent PND utilization was 5.37. Conclusion: The study reflects a very positive attitude of genetically counselled thalassaemia affected families towards PND. For better utilization of PND, genetic counselling services should be available at all health strata. (author)

beta-thalassaemia major hos børn og unge i Danmark

DEFF Research Database (Denmark)

Jung, Anne; Main, Katharina Maria; Scheibel, Elma

2002-01-01

. One patient had died. The body height was between 1.5 and -5.4 SDS (median -1.7) and the sitting height was -0.6 to -5.6 SDS (median -2.3). The bone age was delayed 1-5 years (median -2.5) in six out of ten examined patients, and puberty delayed in four out of five. A dilated left ventricle...... strategy. MATERIAL AND METHODS: During 1998-99 all Danish pediatric departments were contacted for identification of children aged 0-18 years with beta-thalassemia major. Blood transfusions and chelation therapy were registered, and for Eastern Denmark clinical, endocrine, cardiac, and serologic parameters....... An earlier and more effective iron chelation therapy together with improved patient support may reduce growth disturbances and endocrine and cardiac late effects....

Successful application of preimplantation genetic diagnosis for beta-thalassaemia and sickle cell anaemia in Italy.

Science.gov (United States)

Chamayou, S; Alecci, C; Ragolia, C; Giambona, A; Siciliano, S; Maggio, A; Fichera, M; Guglielmino, A

2002-05-01

In Italy, the autosomal recessive diseases beta-thalassaemia and sickle cell anaemia are so widespread that in some regions they can be defined as 'social diseases'. In this study, nine clinical applications of preimplantation genetic diagnosis (PGD) were performed for beta-thalassaemia and sickle cell anaemia on seven Sicilian couples and carriers of beta-globin gene mutations. The studied mutations were: Cd39, HbS, IVS1 nt1, IVS1 nt6 and IVS1 nt110. ICSI was performed with partner's sperm on 131 out of 147 retrieved oocytes, and this resulted in 72 zygotes; 32 embryos were successfully biopsied on day 3. The biopsied blastomeres were lysed and the beta-globin alleles amplified by nested PCR. The mutation diagnosis was performed by restriction enzyme digestion and reverse dot-blot. The amplification efficacy was 97.2%. The genotype study of non-transferred and surplus embryos showed that the allele drop-out rate was 8.6%. Seventeen embryos were transferred in utero on day 4. All couples received an embryo transfer; of the four pregnancies obtained, three resulted in live births and one miscarried at 11 weeks. Prenatal diagnosis at the 11th week and miscarriage material analysis confirmed the PGD results. These studies represent the first successful application of PGD for beta-thalassaemia and sickle cell anaemia in Italy.

The radiological appearances of thalassaemia

Energy Technology Data Exchange (ETDEWEB)

Tyler, P.A. [St Mary' s Hospital, London (United Kingdom)]. E-mail: philippatyler@msn.com; Madani, G. [Royal Free Hospital, London (United Kingdom); Chaudhuri, R. [Whittington Hospital, London (United Kingdom); Wilson, L.F. [Royal National Orthopaedic Hospital, Stanmore (United Kingdom); Dick, E.A. [St Mary' s Hospital, London (United Kingdom)

2006-01-15

The skeletal changes of untreated thalassaemia result from ineffective erythropoiesis and expansion of the bone marrow and affect every part of the skeleton. These changes include osteoporosis, growth retardation, platyspondyly and kyphosis. Erythropoiesis occurs at extra-medullary sites, most commonly resulting in a paraspinal mass but occasionally affecting organs containing pluripotential stem cells. Repeated transfusion unaccompanied by iron chelation causes haemosiderosis; iron is deposited at various sites causing functional impairment. Iron-chelation therapy with desferrioxamine (DFX) prevents haemosiderosis but causes a skeletal dysplasia predominantly affecting the rapidly growing long bones, in particular the distal ulna, and causing irregularity and sclerosis of the physeal-metaphyseal junction and splaying of the metaphysis. DFX also exacerbates the observed growth retardation. DFX-induced skeletal dysplasia may herald toxicity, which is associated with visual and auditory impairment. Therefore, careful balancing of the transfusion regimen and iron-chelation therapy is required. Magnetic resonance imaging (MRI) is the most sensitive technique for the detection of DFX-induced dysplasia.

The radiological appearances of thalassaemia

International Nuclear Information System (INIS)

Tyler, P.A.; Madani, G.; Chaudhuri, R.; Wilson, L.F.; Dick, E.A.

2006-01-01

The skeletal changes of untreated thalassaemia result from ineffective erythropoiesis and expansion of the bone marrow and affect every part of the skeleton. These changes include osteoporosis, growth retardation, platyspondyly and kyphosis. Erythropoiesis occurs at extra-medullary sites, most commonly resulting in a paraspinal mass but occasionally affecting organs containing pluripotential stem cells. Repeated transfusion unaccompanied by iron chelation causes haemosiderosis; iron is deposited at various sites causing functional impairment. Iron-chelation therapy with desferrioxamine (DFX) prevents haemosiderosis but causes a skeletal dysplasia predominantly affecting the rapidly growing long bones, in particular the distal ulna, and causing irregularity and sclerosis of the physeal-metaphyseal junction and splaying of the metaphysis. DFX also exacerbates the observed growth retardation. DFX-induced skeletal dysplasia may herald toxicity, which is associated with visual and auditory impairment. Therefore, careful balancing of the transfusion regimen and iron-chelation therapy is required. Magnetic resonance imaging (MRI) is the most sensitive technique for the detection of DFX-induced dysplasia

An Evaluation of Sensory Neural Hearing Loss in Thalassaemic Patients Treated with Desferrioxamine and Its Risk Factors

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M Sonbolestan

2005-07-01

Full Text Available Back ground: In major thalassaemia patients who need blood transfusion, iron overload is a major therapeutic disadvantage that leads to heart failure which is the major cause of death in such patients. Desferrioxamine (DFO is the most efficient factor for iron chelation, but it carries adverse effects such sensory-neural hearing loss. Methods: The study began in March 2002 and continued untill March 2003, on 160 cases of thalassaemia to determine the incidence of sensory – neural hearing loss and its risk factors in patients who received Desferrioxamine (DFO. All cases underwent audiometric tests. Retrospectively, other needed information were either obtained through interview or extracted from the medical files. Results were analyzed with ANOVA, t-test and Chi-square tests. Results: Seventy-six patients of the total 156 patients showed impairment in PTA (48.7% with 24 of them suffering significant involvement (15.4%. These abnormalities generally affected high frequencies including, 4000 and 8000 Hz. Male gender, increased serum billirubin level and fasting blood sugar were statistically correlated with hearing loss (p.v = 0.038, p.v = 0.38, p.v = 0.002 respectively. There was no significant correlation between hearing loss and other factors. Mean DFO administration in patients, was 29.69 mg/kg/day and mean therapeutic index of DFO was 0.01 mg/kg/day/mg/lit. Both of them were below the critical level (<40mg/kg/day and <0.025mg/kg/day/mg/lit respectively, however hearing loss had developed. Conclusion: Controlling DFO dosage per se does not seem to be enough for decreasing ototoxicity rate. Periodic audiometric tests are highly recommended to detect hearing loss as soon as possible. There are some other factors such as male gender, increased billirubin and FBS, which contribute to DFO ototoxicity. Looking for these risk factors and controlling them, would help identifying susceptible patients and preventing this complication. Key words

Calcium metabolism in children suffering from homozygous β-thalassaemia after oral administration of 47Ca

International Nuclear Information System (INIS)

Liakakos, D.; Vlachos, P.; Anoussakis, C.; Constantinides, C.; Tsakalosos, I.; Alexandra Hospital, Athens

1976-01-01

The study of calcium metabolism in ten thalassaemic children comperatively with controls after oral administration of 47 Ca has shown diminished intestinal absorption. It is suggested that this finding is propably related in part with the pathogenesis of the osteoporosis in thalassaemia. (orig.) [de

Study of a large Anglo-Saxon family with beta-thalassaemia trait.

Science.gov (United States)

Raik, E; Powell, E; Gordon, S

1976-01-01

Study of a large Anglo-Saxon family with beta-thalassaemia trait revealed evidence of consanguinity, moreover both branches of the family shared a Spanish ancestor. The manifestations of the disorder were varied in severity and yet the degree of severity appeared to breed true within any individual part of the family. Our explanation for the inheritance pattern observed in the family was to postulate the existence of two non-allelic genes influencing the rate of beta-chain synthesis.

Estimation of folate binding capacity (unsaturated and total) in normal human serum and in β-thalassaemia

International Nuclear Information System (INIS)

Moulopoulos, S.; Mantzos, J.; Gyftaki, E.; Kesse-Elias, M.; Alevizou-Terzaki, V.; Souli-Tsimili, E.

1978-01-01

A method is described for measuring the total serum folate binding capacity (TBC) after treating the serum with urea at pH5.5, the unsaturated serum folate binding capacity (UBC) being determined without treatment with urea. The method was applied to 50 normal controls and 20 patients with homozygous β-thalassaemia. The results show an increase in folate binding capacity after treating the serum with urea in all cases studied. There is no correlation between serum folic acid level and total or unsaturated folate binding capacity or per cent saturation. The method described is a simple and rapid one for screening the different groups studied for saturated and unsaturated specific folate-binding proteins. (author)

Family reactions and their management in a parents group with beta-thalassaemia.

Science.gov (United States)

Tsiantis, J; Xypolita-Tsantili, D; Papadakou-Lagoyianni, S

1982-11-01

The parents of children with beta-thalassaemia displayed various patterns of emotion (guilt, death anxiety, denial of feelings) and their behaviour towards the child was inappropriate (overprotective, conspiracy of silence); this could affect his psychosocial development and lead to tension within the family. Some parents were overdemanding and even hostile to hospital staff, thus making the management of cases difficult. The therapeutic team has tried to concentrate on these problems in order to clarify them and give support to the families. This has facilitated communication within the family as well as between the family and hospital staff, and had diminished the problems.

Overview of current and emerging issues in endocrinological complications of thalassaemia

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V. de Sanctis de Sanctis

2011-12-01

Full Text Available Clinical advances in the treatment of thalassaemia major (TM patients have helped to increase substantially the life expectancy of patients. The TM patients today represent the first generation of adult thalassemics. As patients enter puberty, they begin to experience a variety of endocrine abnormalities, presumably the results of chronic anaemia and tissue iron deposition from the chronic transfusion therapy. In patients with TM, the anterior pituitary gland is particularly sensitive to free radical stresses. Recent reports have documented a frequency of severe growth hormone deficiency in 13%-32% of adult patients with TM. The prevalence of impaired adrenal function in TM patients has been reported from 0 to 33%, depends on the age of the population studied, although clinical adrenal insufficiency (AI is rare. Thyroid dysfunction has been observed in 13-60% of patients, but its severity is variable in different series. Acquired central hypothyroidism (CH is a rare complication. 治疗重型地中海贫血患者的临床进展已经有助于大幅提高患者的寿命期望。 今天，重型地中海贫血患者代表成年地中海贫血的第一代。 随着病人进入青春期，他们开始经历各种内分泌功能障碍，这些障碍可能是慢性输血治疗导致的慢性贫血和组织铁沉淀结果。 重型地中海贫血患者中，脑下垂体前叶腺对自由基压力尤其敏感。 最近的报告记录了13% - 32% 的重型地中海贫血成年患者严重缺乏生长激素的频率。 尽管临床肾上腺功能不全(AI是十分罕见。但是有报告称重型地中海贫血患者受损的肾上腺皮质功能患病率为0到33%，这个患病率取决于所研究人群的年龄。 对13% - 60%的病人进行了甲状腺机能障碍观察，但是其严重性在不同的系列中不一样。 后天中枢性甲状腺功能减退是一种罕见的并发症。

Emotional assistance in thalassaemia: pilot implementation of a standard protocol

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M.T. Veit

2011-12-01

Full Text Available This study aims to describe the creation process of standard procedures to make possible multicentre studies related to emotional aspects of thalassaemic patients, their families and caregivers; and the pilot phase of the routine implementation. The objectives defined to perform this goal are: i develop routines to assess and manage/treat emotional issues; ii adjust the ABRASTA - Brazilian Association of Thalassaemia computer system to the input of collected data and its compilation; iii conduct a pilot implementation of the routines; iv discuss the whole process and propose next steps. Forty patients were assisted following the above mentioned routines of psychological evaluation, follow-up assistance and management of specific emotional issues. Conclusions are that the routines are adequate to enable multicenter research to compare findings and develop specific interventions to Thalassaemia patients, their families and caregivers; information gathered through them is an important means of supporting medical doctors and other members of the professional team, both in the therapeutic planning and in the communication process with patients and families; finally, considering the nature of the information, psychologists and psychiatrists are the most indicated professionals to perform the assessment and the interventions related to emotional issues, due to their professional background, training and specific skills that allow a free and candid communication with the patients and their families. 本研究旨在描述标准程序的创造过程，来进行关于地中海贫血患者、其家属和照顾者情感方面可能的多中心研究；以及例程实施的试点阶段。 为实现此目的而定下的目标有： 1）制定例程评估和管理/处理情感问题；2）调整巴西地中海贫血病协会（ABRASTA）计算机系统， 输入收集到的数据并对其进行编辑；3）对例程进行试点实施；4�

QUANTITATIVE ULTRASOUND BONE DENSITOMETRY IN CHILDREN WITH THALASSAEMIA IN NORTH EAST INDIA

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Chao Rochek Buragohain

2016-11-01

Full Text Available BACKGROUND Thalassaemic children who are on regular blood transfusion are at increased risk of hypothyroidism, growth hormone deficiency, hypoparathyroidism, diabetes mellitus and osteoporosis because of deposition of iron in various endocrine glands with age. Low bone mineral density is a significant problem in these children, which may lead to increased risk for fractures and suboptimal peak bone mass. The aim of the study is to determine the bone health status of children with thalassaemia using quantitative ultrasound densitometry. MATERIALS AND METHODS A case control study was done at Department of Paediatrics, Jorhat Medical College and Hospital, which included 32 regularly transfused thalassaemic children. Age and sex matched healthy controls were included. Quantitative ultrasound bone densitometry was done in both the groups and compared with each other. RESULTS Broadband Ultrasound Attenuation (BUA and Speed of Sound (SOS measurements were found to be independent of sex. BUA values in boys showed increasing trend with age, which was not observed in girls. SOS values did not show any increasing trend with age in both sexes. In both the groups, the BUA and SOS increased continuously with increasing age. The values of BUA were more in control group compared to case group while opposite was noticed with SOS values. BUA values were more when ferritin level was >2000 ng/dL and increased number of blood transfusion, whereas SOS values decreased with increase in serum ferritin level and number of transfusion, which was statistically significant (P=0.0125. Both BUA and SOS values decreased with increased severity of malnutrition, which was statistically significant for SOS (P=0.0266. CONCLUSION Quantitative ultrasound bone densitometry can be used as a screening method to assess the bone health status in children, particularly those with thalassaemia, but requires further studies in large groups.

Red cell 2,3-diphosphoglycerate levels in children with hereditary haemolytic anaemias.

Science.gov (United States)

Haidas, S; Zannos-Mariolea, L; Matsaniotis, N

1975-12-01

The role of red cell 2,3-diphosphoglycerate (2,3-DPG) in increasing the availability of haemoglobin oxygen in neonatal jaundice and hereditary haemolytic anaemias was investigated. Measurements of 2,3-DPG were carried out on 58 normal children and six normal adults, 18 full-term newborns with neonatal jaundice and 57 cases (51 children and six adults) with hereditary haemolytic anaemias. In normal children and adults, with a mean haemoglobin of 12.69 g/dl, mean 2,3-DPG was 14.90 mumol/g Hb. In jaundiced newborns with a mean haemoglobin of 16.04 g/dl mean 2,3-DPG levels were 14.51 mumol/g Hb, i.e. normal. 2,3-DPG levels were increased in patients with beta-thalassaemia major, alpha-thalassaemia, sickle-cell disease, favism, hereditary spherocytosis and in heterozygotes for beta-thalassaemia with increased haemoglobin F. In heterozygotes for beta-thalassaemia with increased haemoglobin A2 only and in sickle cell trait 2,3-DPG levels were normal.

Care for Haemoglobinopathy Patients in Slovakia.

Science.gov (United States)

Fábryová, Viera; Božek, Peter; Drakulová, Monika; Kollárová, Andrea; Striežencová, Zuzana Laluhová; Macichová, Michaela; Sakalová, Adriena

2017-03-01

The paper presents the results od 22-year study of screening and follow-up of haemoglobinopathies in Slovakia, an overview of genetic mutations, the coincidence with hereditary haemochromatosis mutations, and the procedure in genetic councelling. Between 1993-2015, in three centres in Bratislava and in one centre in Kosice, carriers of beta-thalassaemic genes or other haemoglobinopathies were searched for. Diagnosis was performed by haematologists, whereby the family history was evaluated, together with the overall clinical condition, blood count and blood smear, iron and haemolysis parameters, mutations of hereditary haemochromatosis, and haemoglobin electrophoresis testing. In the last years the haemoglobin division also examined by high performance liquid chromatography (HPLC). A clinical suspicion of the heterozygous form of beta-thalassaemia or other haemoglobinopathies was documented in 554 patients. Of them 32 (5.8%) were foreigners. 213 (38.45%) patients were genetically examined. In 190 (33.93%) of them heterozygote beta-thalassaemia was confirmed. The most frequent mutations were IVS 1.110 (33.15%), IVS 2.1 (33.15%), and IVS 1.6 (14.7%). Evidence of haemoglobin S (heterozygote sickle cell anaemia) was also notable in two non-relative children, whose fathers were of African origin, and one patient from Ghana. One female patient was followed up for haemoglobin Santa Ana (non-stabile haemoglobin previously diagnosed as mutation de novo). In our group, we took care of pregnant patients with haemoglobinopathies. The study showed that there is a higher number of heterozygotes for beta-thalassaemia and rarely haemoglobinopathies in Slovakia. Over the past years, we have recorded an increase number of foreigners coming to our country. It is necessary to continue in search of pathological gene carriers to avoid serious forms of haemoglobinopathies. Copyright© by the National Institute of Public Health, Prague 2017

Burden, genotype and phenotype profiles of adult patients with ...

African Journals Online (AJOL)

) a cross-sectional analysis of clinical and haematological characteristics of SCD patients; and (iii) molecular analysis of the haemoglobin S mutation, the haplotype in the β-globinlike genes cluster, the 3.7 kb α-thalassaemia gene deletion and ...

Bone disease in haemoglobin disorders

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Ersi Voskaridou

2013-03-01

Full Text Available Bone disease represents a prominent cause of morbidity in patients with thalassaemia and other haemoglobin disorders. The delay in sexual maturation, the presence of diabetes and hypothyroidism, the parathyroid gland dysfunction, the haemolytic anaemia, the progressive marrow expansion, the iron toxicity on osteoblasts, the iron chelators, and the deficiency of growth hormone or insulin growth factors have been identified as major causes of osteoporosis in thalassaemia. Adequate hormonal replacement, effective iron chelation, improvement of hemoglobin levels, calcium and vitamin D administration, physical activity, and smoking cessation are the main to-date measures for the management of the disease. During the last decade, novel pathogenetic data suggest that the reduced osteoblastic activity, which is believed to be the basic mechanism of bone loss in thalassemia, is accompanied by a comparable or even greater increase in bone resorption. Therefore, potent inhibitors of osteoclast activation, such as the aminobisphosphonates, arise as key drugs for the management of osteoporosis in thalassaemia patients and other haemoglobin disorders.

Awareness among Parents of β-Thalassemia Major Patients Regarding Prenatal Diagnosis and Premarital Screening in Day Care Centre of Transfusion Medicine Department.

Science.gov (United States)

Rudra, S; Chakrabarty, P; Hossain, M A; Ripon, M J; Rudra, M; Mirza, T T

2016-01-01

Thalassemia is one of the most common genetic diseases in the world. It is a major health problem, brings much morbidity, early mortality and a great deal of misery for a family both financially and emotionally. The patients suffering from beta thalassemia major do not survive for more than 5 years without blood transfusion. Blood transfusion is usually administered every two to five weeks to maintain the pre-transfusion hemoglobin level of 9-10 gm/dL. This study carried out in the department of Transfusion Medicine of Mymensingh Medical College Hospital from January 2014 to June 2014. A total of 200 parents were interviewed. There was a slight preponderance of females which accounted for 57.5% of the parents. Ninety seven (45.5%) had an income less than Rs. 5000 per month. Nearly 50% were illiterate with only 24.5% with a higher education. Consanguinity was positive in 72.5% of the parents with extended family history of thalassemia positive in 40.8%. Only 29.5% were immunized against Hepatitis B. Around 27.5% did not know whether they should be immunized. Fifty five percent of parents knew children should receive Dysferol. Twelve percent were aware of consanguinity to be a risk factor for thalassaemia with only 5% having undergone antenatal diagnosis. Parental knowledge about thalassemia and its preventive measures is inadequate; this requires intervention in the form of public health education programs concentrating on high risk/targeted population.

Magnetic resonance imaging of marrow heterotopia in haemoglobinopathy

Energy Technology Data Exchange (ETDEWEB)

Papavasiliou, C.; Trakadas, S.; Gouliamos, A.; Vlahos, L.; Pouliadis, G.; Phessas, P.

1988-02-01

The MRI findings of masses of MH in three patients suffering from thalassaemia major, sickle-cell anaemia and thalassaemia intermedia, respectively, are presented. There was good correlation between the MRI findings and the conventional radiology of CT appearance of the masses. The masses produced a low intensity signal, similar to that of the adjacent marrow of the thoracic spine, while they were surrounded by a high-intensity signal rim attributed to a layer of fat surrounding the masses. The latter MRI finding is thought to be characteristic of MH masses.

Magnetic resonance imaging of marrow heterotopia in haemoglobinopathy

International Nuclear Information System (INIS)

Papavasiliou, C.; Trakadas, S.; Gouliamos, A.; Vlahos, L.; Pouliadis, G.; Phessas, P.

1988-01-01

The MRI findings of masses of MH in three patients suffering from thalassaemia major, sickle-cell anaemia and thalassaemia intermedia, respectively, are presented. There was good correlation between the MRI findings and the conventional radiology of CT appearance of the masses. The masses produced a low intensity signal, similar to that of the adjacent marrow of the thoracic spine, while they were surrounded by a high-intensity signal rim attributed to a layer of fat surrounding the masses. The latter MRI finding is thought to be characteristic of MH masses. (orig.)

World health dilemmas: Orphan and rare diseases, orphan drugs and orphan patients.

Science.gov (United States)

Kontoghiorghe, Christina N; Andreou, Nicholas; Constantinou, Katerina; Kontoghiorghes, George J

2014-09-26

According to global annual estimates hunger/malnutrition is the major cause of death (36 of 62 million). Cardiovascular diseases and cancer (5.44 of 13.43 million) are the major causes of death in developed countries, while lower respiratory tract infections, human immunodeficiency virus infection/acquired immunodeficiency syndrome, diarrhoeal disease, malaria and tuberculosis (10.88 of 27.12 million) are the major causes of death in developing countries with more than 70% of deaths occurring in children. The majority of approximately 800 million people with other rare diseases, including 100000 children born with thalassaemia annually receive no treatment. There are major ethical dilemmas in dealing with global health issues such as poverty and the treatment of orphan and rare diseases. Of approximately 50000 drugs about 10% are orphan drugs, with annual sales of the latter approaching 100 billion USD. In comparison, the annual revenue in 2009 from the top 12 pharmaceutical companies in Western countries was 445 billion USD and the top drug, atorvastatin, reached 100 billion USD. In the same year, the total government expenditure for health in the developing countries was 410 billion USD with only 6%-7% having been received as aid from developed countries. Drugs cost the National Health Service in the United Kingdom more than 20 billion USD or 10% of the annual health budget. Uncontrollable drug prices and marketing policies affect global health budgets, clinical practice, patient safety and survival. Fines of 5.3 billion USD were imposed on two pharmaceutical companies in the United States, the regulatory authority in France was replaced and clinicians were charged with bribery in order to overcome recent illegal practises affecting patient care. High expenditure for drug development is mainly related to marketing costs. However, only 2 million USD was spent developing the drug deferiprone (L1) for thalassaemia up to the stage of multicentre clinical trials. The

World health dilemmas: Orphan and rare diseases, orphan drugs and orphan patients

Science.gov (United States)

Kontoghiorghe, Christina N; Andreou, Nicholas; Constantinou, Katerina; Kontoghiorghes, George J

2014-01-01

According to global annual estimates hunger/malnutrition is the major cause of death (36 of 62 million). Cardiovascular diseases and cancer (5.44 of 13.43 million) are the major causes of death in developed countries, while lower respiratory tract infections, human immunodeficiency virus infection/acquired immunodeficiency syndrome, diarrhoeal disease, malaria and tuberculosis (10.88 of 27.12 million) are the major causes of death in developing countries with more than 70% of deaths occurring in children. The majority of approximately 800 million people with other rare diseases, including 100000 children born with thalassaemia annually receive no treatment. There are major ethical dilemmas in dealing with global health issues such as poverty and the treatment of orphan and rare diseases. Of approximately 50000 drugs about 10% are orphan drugs, with annual sales of the latter approaching 100 billion USD. In comparison, the annual revenue in 2009 from the top 12 pharmaceutical companies in Western countries was 445 billion USD and the top drug, atorvastatin, reached 100 billion USD. In the same year, the total government expenditure for health in the developing countries was 410 billion USD with only 6%-7% having been received as aid from developed countries. Drugs cost the National Health Service in the United Kingdom more than 20 billion USD or 10% of the annual health budget. Uncontrollable drug prices and marketing policies affect global health budgets, clinical practice, patient safety and survival. Fines of 5.3 billion USD were imposed on two pharmaceutical companies in the United States, the regulatory authority in France was replaced and clinicians were charged with bribery in order to overcome recent illegal practises affecting patient care. High expenditure for drug development is mainly related to marketing costs. However, only 2 million USD was spent developing the drug deferiprone (L1) for thalassaemia up to the stage of multicentre clinical trials. The

Single Breath-Hold Physiotherapy Technique; Effective tool for T2* magnetic resonance imaging in young patients with thalassaemia major

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Surekha T. Mevada

2016-02-01

Full Text Available Magnetic resonance imaging using T2* (MRI T2* is a highly sensitive and non-invasive technique for the detection of tissue iron load. Although the single breath-hold multi-echo T2* technique has been available at the Sultan Qaboos University Hospital (SQUH, Muscat, Oman, since 2006, it could not be performed on younger patients due to their inability to hold their breath after expiration. This study was carried out between May 2007 and May 2015 and assessed 50 SQUH thalassaemic patients aged 7‒17 years old. Seven of these patients underwent baseline and one-year follow-up MRI T2* scans before receiving physiotherapy training. Subsequently, all patients were trained by a physiotherapist to hold their breath for approximately 15‒20 seconds at the end of expiration before undergoing baseline and one-year follow-up MRI T2* scans. Failure rates for the pre- and post-training groups were 6.0% and 42.8%, respectively. These results indicate that the training of thalassaemic patients in breathhold techniques is beneficial and increases rates of compliance for MRI T2* scans.

The Prevalence of the Beta Thalassemia Trait among the Pregnant Women who attended the ANC Clinic in a PHC, by using the NESTROF Test in Bangalore, Karnataka.

Science.gov (United States)

Kulkarni, Praveen; Masthi, N R Ramesh; Niveditha, Sr; Suvarna, R

2013-07-01

Contaxt: Every year in India 6000 to 8000 children are born with thalassaemia major. The birth of such a child produces considerable physical and economic strain on the affected child, its family and the community at large. Thus, the emphasis must shift from the treatment to the prevention of such births in the future. To find out the prevalence of the Beta Thalassaemia trait among the pregnant women who attended the antenatal clinics in a Primary Health Centre, by using the NESTROF test; to describe the socio-demographic characteristics of the study subjects, to find out the pregnancies which were 'at risk' of delivering babies with Thalassaemia major and to find out the 'awareness' of the pregnant women regarding Thalassaemia. This exploratory study was conducted in a PHC which was attached to the Department of Community Medicine of a medical college which was situated in Bangalore, India, for a period of 3 months. All the pregnant women who attended the antenatal clinic and the husbands of the NESTROF positive women were included in the study. The details regarding the sociodemographic characteristics of the women were collected on a structured proforma and the NESTROF test was performed. Out of the 210 pregnant women who were tested, 18 (8.5%) were thalassaemia carriers. 12 (66.6%) of them were between 20 - 25 years of age. 5 (27.7%) were born out of 2(nd) degree consanguineous marriages. 7 (38.8%) had a history of abortions, among which 6 (33.3%) were in the 1(st) trimesters of their pregnancies. Out of the 18 positive women, 9 (50%) had turned up with their husbands. All of the husbands were negative for the Thalassaemia carrier status. Thus, there was no pregnancy which was at a risk of delivering babies with thalassaemia major. None (100%) of the pregnant women were aware of the disease, thalassaemia. The prevalence of the Beta Thalassaemia trait among the pregnant women was 8.5%.

Major life events and development of major depression in Parkinson's disease patients

DEFF Research Database (Denmark)

Rod, Naja Hulvej; Bordelon, Y; Thompson, A

2012-01-01

BACKGROUND AND PURPOSE: Non-motor symptoms including depression are important features of Parkinson's disease (PD). We aim to address the relationship between major life events and depression amongst PD patients free of depressive symptoms at baseline. METHODS: New-onset PD patients from California...... were recruited in 2001-2007 and followed up for 3-4 years. The participants (n = 221) were examined by neurologists and responded to comprehensive interviews that included major life events, social support, and coping measures from validated scales. Major depression was assessed using the Structured...... Clinical Interview for the DSM-IV depression module (SCID). RESULTS: More than half of all patients had experienced major life events since diagnosed with PD, and 22 patients developed a major depression. The number of life events was associated with risk of depression in an exposure-dependent manner...

Non cardiopatic and cardiopatic beta thalassaemic patients: quantitative and qualitative cardiac iron deposition evaluation with MRI

International Nuclear Information System (INIS)

Macarini, Luca; Marini, Stefania; Scardapane, Arnaldo; Pietrapertosa, Anna; Ettore, Giovanni Carlo

2005-01-01

Purpose: Cardiomyopathy is one of the major complications of β thalassaemia major as a result of transfusion iron overload. The aim of our study is to evaluate with MR if there is any difference of iron deposition signal intensity (SI) or distribution between non-cardiopatic and cardiopatic thalassaemic patients in order to establish if there is a relationship between cardiopathy and iron deposition. Materials and methods: We studied 20 patients affected by β thalassaemia major, of whom 10 cardiopatic and 10 non-cardiopatic, and 10 healthy volunteers as control group. Serum ferritin and left ventricular ejection fraction were calculated in thalassaemic patients. All patients were examinated using a 1.5 MR unit with ECG-gated GE cine-MR T2*-weighted, SE T1-weighted and GE T2*-weighted sequences. In all cases, using an adequate ROI, the myocardial and skeletal muscle signal intensity (SI), the myocardial/skeletal muscle signal intensity radio (SIR) and the SI average of the myocardium and skeletal muscle were calculated for every study group. The qualitative evaluation of iron deposition distribution was independently performed by three radiologists who analysed the extension, the site and the morphology of iron deposition on the MR images and reported their observations on the basis of a four-level rating scale: 0 (absent), 1 (limited), 2 (partial), 3 (widespread deposition). The results of quantitative and qualitative evaluation were analysed with statistical tests. Results: Cardiac iron deposition was found in 8/10 non-cardiopatic thalassaemic patients and in all cardiopatic thalassaemic patients. We noticed a significant SI difference (p>0.05) between the healthy volunteer control group and the thalassaemic patients with iron deposition, but no significant SI difference in iron deposition between non-cardiopatic thalassaemic patients in the areas evaluated. The qualitative evaluation revealed a different distribution of iron deposition between the two

[Clinical management of beta-thalassaemia].

Science.gov (United States)

Thuret, Isabelle

2014-10-01

Beta-thalassemia syndromes are autosomal recessive disorders related to the inability to produce beta-globin chains. Thalassemia major is by definition a transfusion dependent anemia and iron overload is the leading cause of death and morbidity. Beta-thalassemia is rarely encountered in France where patients mainly originated from Mediterranean countries and South East Asia. Recently, two major advances have substantially improved the disease management: oral iron chelation therapy and the introduction of cardiac MRI for monitoring cardiac iron. Hematopoietic stem cell transplantation remains, in clinical practice, the only curative approach and is proposed to children having an HLA-identical sibling. Diagnosis of thalassemia trait is important in order to propose genetic counseling to couples at risk. Thalassemia intermedia is a clinical entity where anemia is mild or moderate, requiring no or occasional transfusion. Clinical severity increases with age with a more severe anemia, thrombotic complications and extra-medullary hematopoiesis. Iron overload, optimally monitored with liver MRI, occurs in adult patients and is related to increased iron hyper-absorption.

The impact of major trauma network triage systems on patients with major burns.

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Nizamoglu, Metin; O'Connor, Edmund Fitzgerald; Bache, Sarah; Theodorakopoulou, Evgenia; Sen, Sankhya; Sherren, Peter; Barnes, David; Dziewulski, Peter

2016-12-01

Trauma is a leading cause of death and disability worldwide. Patients presenting with severe trauma and burns benefit from specifically trained multidisciplinary teams. Regional trauma systems have shown improved outcomes for trauma patients. The aim of this study is to determine whether the development of major trauma systems have improved the management of patients with major burns. A retrospective study was performed over a four-year period reviewing all major burns in adults and children received at a regional burns centre in the UK before and after the implementation of the regional trauma systems and major trauma centres (MTC). Comparisons were drawn between three areas: (1) Patients presenting before the introduction of MTC and after the introduction of MTC. (2) Patients referred from MTC and non-MTC within the region, following the introduction of MTC. (3) Patients referred using the urban trauma protocol and the rural trauma protocol. Following the introduction of regional trauma systems and major trauma centres (MTC), isolated burn patients seen at our regional burns centre did not show any significant improvement in transfer times, admission resuscitation parameters, organ dysfunction or survival when referred from a MTC compared to a non-MTC emergency department. There was also no significant difference in survival when comparing referrals from all hospitals pre and post establishment of the major trauma network. No significant outcome benefit was demonstrated for burns patients referred via MTCs compared to non-MTCs. We suggest further research is needed to ascertain whether burns patients benefit from prolonged transfer times to a MTC compared to those seen at their local hospitals prior to transfer to a regional burns unit for further specialist care. Copyright © 2016 Elsevier Ltd and ISBI. All rights reserved.

Diabetes mellitus in β-thalassemia major patients

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Riadi Wirawan

2003-06-01

Full Text Available β-thalassemia major is a disease caused by β polypeptide chain synthesis disorder which is inherited as an autosomal recessive from both parents which is marked by little or no β globin chain synthesis. Medication for β thalassemia major patients is by repeated blood transfusions, which causes hemochromatosis. Hemochromatosis can occur in various organs including the pancreas. The aim of the study was to assess the alteration of plasma glucose concentration and the hemochromatosis prevalence. Fasting plasma glucose concentration and serum ferritin examination were measured in 115 β thalassemia major patients with ages between 10-23 years who were out-patients in the Thalassemia Centre, Department of Child Health, Medical School, University of Indonesia / Dr. Cipto Mangunkusumo General Hospital, Jakarta. The plasma glucose concentration examination was conducted by the GDH enzymatic method, with American Diabetes Association (ADA criteria in the evaluation, while the serum ferritin examination was conducted with the microparticle enzyme immuno assay (MEIA method. All patients had hemochromatosis, 14.8% of the patients had impaired fasting glucose level and 2.6% of the patients showed indications of diabetes mellitus. β thalassemia major patients who receive frequent transfusions will develop hemochromatosis that will in turn impair the pancreatic function. (Med J Indones 2003; 12: 87-93 Keywords : β thalassemia major, hemochromatosis, diabetes mellitus

Endocrinopathies in thalassemia major patient

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Lubis, D. A.; Yunir, E. M.

2018-03-01

Advanced in chelation therapy and regular blood transfusion have marked improvements in the life expectancy of patients with thalassemia major, however these patients still have to deal with several complications. We report a 19-year-old male, presented with multiple endocrine complication-related thalassemia; hypogonadism, short stature, osteoporosis with history of fracture, and subclinical hypothyroid.

Endocrine investigation and follow up in thalassaemia. Time for specific guidelines

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N. Skordis

2011-12-01

Full Text Available Iron overload due to multiple transfusions affects the endocrine glands especially the anterior pituitary, the pancreas, the thyroid and the parathyroids. This leads to a variety of endocrinopathies and growth failure. Delayed puberty, hypogonadism, growth hormone deficiency in adults, hypothyroidism, hypoparathyroidism and diabetes are common and around 20% of patients have more than one endocrinopathy. In this paper suggestions for guidelines concerning diagnosis, investigation and treatment are proposed for the following clinical entities encountered in thalassaemia patients: i Growth failure: after the age of 9-10 rears there is a slowing of growth velocity, the pathogenesis of which is multifactorial and anaemia, folate deficiency and hypersplenism are implicated. Desferrioxamine toxicity has been reported as cause of the abnormal upper to lower segment ratio. Growth hormone is given in selected cases. ii Delayed puberty and hypogonadism: are the most obvious clinical consequences of iron overload in both sexes. Primary and secondary amenorrhoea are very common in women. Sex steroid replacement therapy is the optimal therapeutic regime which has a great impact on the quality of life of adult thalassaemia patients. iii Fertility: Women with TM, who are regularly transfused and are well chelated can now become pregnant either spontaneously or by inducing ovulation. Pregnancy must carefully monitored. iv Growth hormone deficiency in adult thalassaemics: This occurs in a high prevalence and since GH in adults is involved in numerous biological functions, especially of the heart, proper assessment of this hormone is needed and consideration of the need for replacement. v Hypothyroidism and hypoparathyroidism: these deficiencies are also discussed. 由于多次输血引起的铁过载影响内分泌腺，尤其是垂体前叶、胰腺、甲状腺以及甲状旁腺 这导致各种内分泌病和成长失败。 �

Establishing a legal service for major trauma patients at a major trauma centre in the UK.

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Seligman, William H; Thompson, Julian; Thould, Hannah E; Tan, Charlotte; Dinsmore, Andrew; Lockey, David J

2017-09-01

Major trauma causes unanticipated critical illness and patients have often made few arrangements for what are sudden and life-changing circumstances. This can lead to financial, housing, insurance, legal and employment issues for patients and their families.A UK law firm worked with the major trauma services to develop a free and comprehensive legal service for major trauma patients and their families at a major trauma centre (MTC) in the UK. In 2013, a legal service was established at North Bristol NHS Trust. Referrals are made by trauma nurse practitioners and it operates within a strict ethical framework. A retrospective analysis of the activity of this legal service between September 2013 and October 2015 was undertaken. 66 major trauma patients were seen by the legal teams at the MTC. 535 hours of free legal advice were provided on non-compensation issues-an average of 8 hours per patient. This initiative confirms a demand for the early availability of legal advice for major trauma patients to address a range of non-compensation issues as well as for identification of potential compensation claims. The availability of advice at the MTC is convenient for relatives who may be spending the majority of their time with injured relatives in hospital. More data are needed to establish the rehabilitation and health effects of receiving non-compensation advice after major injury; however, the utilisation of this service suggests that it should be considered at the UK MTCs. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Mathematical analysis of 51Cr-labelled red cell survival curves in congenital haemolytic anaemias

International Nuclear Information System (INIS)

Kasfiki, A.G.; Antipas, S.E.; Dimitriou, P.A.; Gritzali, F.A.; Melissinos, K.G.

1982-01-01

The parameters of 51 Cr labelled red cell survival curves were calculated in 26 patients with homozygous β-thalassaemia, 8 with sickle-cell anaemia and 3 with s-β-thalassaemia, using a non-linear weighted least squares analysis computer program. In thalassaemic children the calculated parameters denote that the shorting of the mean cell life is due to early senescence alone, while there is some evidence that in thalassaemic adults additional extracellular destruction mechanisms participate as well. Red cell survival curves from patients with sickle-cell anaemia and s-β-thalassaemia resemble each other, while their parameters indicate an initial rapid loss of radioactivity, early senescence and the presence of extracellular red cell destruction factors. (orig.)

Retinal detachment associated with thalassemia major and Marfan′s syndrome-A case report

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Chatterjee Pranab

1987-01-01

Full Text Available The thalassaemias, unlike a few other disorders of the haemopoietic system, are very rarely accompanied by ophthalmological changed. Marfan′s Syndrome, on the other hand, exhibits a number of ocular manifestations 2. This article describes a case of uniocular retinal detachment associated with both these conditions

Body Composition in Adult Patients with Thalassemia Major

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Marianna Vlychou

2016-01-01

Full Text Available Objective. To assess body composition in adult male and female patients with thalassemia major by dual-energy X-ray absorptiometry (DXA and to compare the findings with a group of healthy age-matched controls. Methods. Our study group included sixty-two patients (27 males, mean age 36 years, and 35 females, mean age 36.4 years and fifteen age-matched healthy controls. All patients had an established diagnosis of thalassemia major and followed a regular blood transfusion scheme since childhood and chelation treatment. Fat, lean, and bone mineral density (BMD were assessed with dual-energy X-ray absorptiometry. Ferritin levels and body mass index of all patients and controls were also recorded. Student t-test and Wilcoxon test were performed and statistical significance was set at p<0.05. Results. BMD and whole body lean mass are lower in both male and female adult patients compared with controls (p<0.01 in both groups, whereas whole body fat mass was found to have no statistically significant difference compared to controls. Regional trunk fat around the abdomen was found to be lower in male patients compared to controls (p=0.02. Conclusion. Severe bone loss and diminished lean mass are expected in adult male and female patients with thalassemia major. Fat changes seem to affect mainly male patients.

The patient perspective in research on major depression

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Cuijpers Pim

2011-05-01

Full Text Available Abstract Although thousands of studies have examined the genetics, epidemiology, etiology, biology, treatment and prevention of major depressive disorder, we still lack very basic knowledge about what patients with depressive disorders need. Despite the thousands of studies that have been conducted on major depression and the hundreds of randomized trials that have examined the effects of treatments, many patients still do not know how to cope with the daily problems caused by depressive disorders. In this Commentary the need for more research on the perspectives of patients is described. This research should guide treatment studies as well as basic research much more than it currently does. This perpective is especially important to understand and solve the undertreatment of depression, one of the major problems in this area. Up to 50% of depressed patients do not seek treatment, resulting in huge avoidable disease burden and economic costs. In order to solve this problem we need a better understanding of the problems patients encounter in daily life, and what factors contribute to the reasons for seeking treatment or not. Research from the patients' perspective is also necessary to meet the currently unmet information needs of patients, including information about the nature and causes of depression, stigma, medication, treatment and coping with the daily problems of having depression.

DOES INSULIN LIKE GROWTH FACTOR-1 (IGF-1 DEFICIENCY HAVE A “PROTECTIVE” ROLE IN THE DEVELOPMENT OF DIABETIC RETINOPATHY IN THALASSEMIA MAJOR PATIENTS?

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Vincenzo De Sanctis

2015-05-01

Full Text Available Objective: This cross-sectional study was designed to give insights into relationship between Insulin-Growth-Factor 1 (IGF-1 levels and diabetic retinopathy (DR in a sample of  thalassaemia major(TM patients with insulin dependent diabetes mellitus (IDDM. Τhis relation was not previously evaluated, despite the fact that both diseases co-exist  in the same patient. The  study   also  describes the clinical and biochemical profile of the associated complications in TM patients with and without IDDM.    Design: A population-based cross-sectional study.   Participants:The study  includes 19 consecutive TM patients with IDDM and 31 age- and sex-matched TM patients without  IDDM who visited our out-patient clinics for an endocrine assessment   Methods: An extensive medical history, with data on associated complications and current medications, was obtained. Blood samples were drawn in the morning after an overnight fast to measure the serum concentrations of IGF-1, glucose, fructosamine , free thyroxine (FT4, thyrotropin (TSH and biochemical analysis . Serologic screening assays for hepatitis C virus seropositivity (HCVab and HCV-RNA were also evaluated,  applying routine laboratory methods.Plasma total IGF-1 was measured by a chemiluminescent immunometric assay (CLIA method. Ophthalmology evaluation was done by the same researcher using stereoscopic fundus biomicroscopy through dilated pupils. DR was graded using the scale developed by the Global Diabetic Retinopathy Group. Iron stores were assessed by direct and indirect methods.   Results:Eighteen TM patients with IDDM (94.7 % and 10 non-diabetic patients (32.2 % had IGF-1 levels below the 2.5th percentile of the normal values for the Italian population. The mean serum IGF-1 concentrations were significantly lower in the diabetic versus the non-diabetic TM groups (p < 0.001. DR was present in in 4 (21 % of 19 TM patients with IDDM and was associated with the main classical risk

Haemoglobinopathies in Greece: prevention programme over the past 35 years.

Science.gov (United States)

Loukopoulos, Dimitris

2011-10-01

At present, prevention of thalassaemia and sickle cell disease is the only realistic approach to control the birth of new patients in countries having high numbers of carriers. This is fully justified because avoiding the birth of an ever increasing number of patients may allow a more effective use of the available resources in improving the management of the patients surviving today and alleviate the already overloaded public health system from the inevitable tremendous and ever increasing cost. Moreover, prenatal diagnosis may help couples at risk to have non-thalassaemic children. Greece is one of the countries where the mean frequency of carriers is approximately 7.5 per cent (population 11 million) and has set up a nationwide programme for carrier identification in the early seventies; this is provided through a dozen of specific Units attached to the major Blood Transfusion Services of the country, on a voluntary basis and free of charge. Spread of information through mass media, the schools, and other groups has greatly contributed in creating the necessary sensitization; obstetricians and antenatal Clinics are also instrumental to this effect. Prenatal diagnosis is offered centrally (Athens) and covers satisfactorily the estimated needs (500-600 annually); the total number has already exceeded 35,000. According to information obtained from the major paediatric hospitals all over the country, the number of thalassaemia major or SCD admitted for treatment over the last ten years has been around 15 yearly (instead of an estimate of 120-130).

Mental health status in patients with Thalassemia major in Iran

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Mahdieh Nasiri

2014-02-01

Full Text Available Thalassemia major is a genetic blood disorder that is detected by the symptoms of chronic and severe anemia, enlarged liver and spleen, failure to thrive and bone deformities in particular deformed face and bulging forehead. Due to changes in physical appearance, the disease can influence on other aspects of the patient's life, so the disease can have a strong impact on the mental health of these patients and their families. Previous studies showed that 80% of patients with thalassemia major have at least one psychiatric disorder. The aim of this paper was to review the mental health status of patients with Beta-thalassemia major in Iran.

The patient perspective in research on major depression

NARCIS (Netherlands)

Cuijpers, P.

2011-01-01

Although thousands of studies have examined the genetics, epidemiology, etiology, biology, treatment and prevention of major depressive disorder, we still lack very basic knowledge about what patients with depressive disorders need. Despite the thousands of studies that have been conducted on major

Frequency of short stature in - thalassemia major patients

International Nuclear Information System (INIS)

Aslam, M.S.; Roshan, E.; Shahid, M.

2013-01-01

Objective: To determine the frequency of short stature in children with - thalassemia major receiving multiple transfusions at Military Hospital Rawalpindi. Study Design: Descriptive cross sectional study. Place and Duration of Study: Study was conducted at Military Hospital from 1st January 2010 to 30th June 2010. Subjects and Methods: Total 100 multi-transfused cases of - thalassemia major were included in the study. The height of every child was measured in centimeters using the same free-standing standard stadiometer, and the same technique by a single pediatrician. Results: Out of 100 patients of - thalassemia major 57.0% (n=57) were male while 43% (n=43) were female. Mean age was 9.94 years (SD +- 2.93) with range of 6 to 14 years. Mean height was 115.77 cm (SD +- 13.79) with range of 72.00 to 148.00 cm. 57.0% (n=57) were found to be short statured while 43.0% (n=43) were with normal height. Mean age of short statured patients was 11.61 +- 2.34 years and mean age of patients with normal height was 7.73 +- 2.05 years. Conclusion: The frequency of short stature in our patients with - thalassemia major receiving multiple transfusions is high. There is need to monitor the height of thalassemic children regularly and to improve the quality of care being provided to them so as to improve their quality of life. (author)

Thalassaemia, today

International Nuclear Information System (INIS)

Scutellari, P.N.; Orzincolo, C.; Bagni, B.; Franceschini, F.; Atti, G.

1988-01-01

The main abnormalities of the hand and skull in a group of patients who underwent hypertransfusion regimen were reviewed and compared to the lesions observed in a control group of low-transfused patients. The results are reported, and a possible correlation with transfusions regimen is discussed: since bone lesions are explained on the basis of relationship between proliferating bone marrow and bone cortex, hypertransfusion regimen will prevent the development of abnormalities, only if established early in life. For this reason, skeletal lesions in Cooley's anaemia have changet their ''classical'' radiography patterns. In the skull, the diploic space may become normal, and the overgrowth of facial bones moderate; the pneumatization of paranasal sinuses may not be completely impeded by the swelling of the jaws, the ''hair-brush'' pattern may disappear completely. The normalized picture of the hand is and remains a typical differential sign of prepuberal and adult patients

Measurement of HbA1c and HbA2 by Capillarys 2 Flex Piercing HbA1c programme for simultaneous management of diabetes and screening for thalassemia.

Science.gov (United States)

Ke, Peifeng; Liu, Jiawei; Chao, Yan; Wu, Xiaobin; Xiong, Yujuan; Lin, Li; Wan, Zemin; Wu, Xinzhong; Xu, Jianhua; Zhuang, Junhua; Huang, Xianzhang

2017-10-01

Thalassemia could interfere with some assays for haemoglobin A 1c (HbA 1c ) measurement, therefore, it is useful to be able to screen for thalassemia while measuring HbA 1c . We used Capillarys 2 Flex Piercing (Capillarys 2FP) HbA 1c programme to simultaneously measure HbA 1c and screen for thalassemia. Samples from 498 normal controls and 175 thalassemia patients were analysed by Capillarys 2FP HbA 1c programme (Sebia, France). For method comparison, HbA 1c was quantified by Premier Hb9210 (Trinity Biotech, Ireland) in 98 thalassaemia patients samples. For verification, HbA 1c from eight thalassaemia patients was confirmed by IFCC reference method. Among 98 thalassaemia samples, Capillarys 2FP did not provide an HbA 1c result in three samples with HbH due to the overlapping of HbBart's with HbA 1c fraction; for the remaining 95 thalassaemia samples, Bland-Altman plot showed 0.00 ± 0.35% absolute bias between two systems, and a significant positive bias above 7% was observed only in two HbH samples. The HbA 1c values obtained by Capillarys 2FP were consistent with the IFCC targets (relative bias below ± 6%) in all of the eight samples tested by both methods. For screening samples with alpha (α-) thalassaemia silent/trait or beta (β-) thalassemia trait, the optimal HbA 2 cut-off values were ≤ 2.2% and > 2.8%, respectively. Our results demonstrated the Capillarys 2FP HbA 1c system could report an accurate HbA 1c value in thalassemia silent/trait, and HbA 2 value (≤ 2.2% for α-thalassaemia silent/trait and > 2.8% for β-thalassemia trait) and abnormal bands (HbH and/or HbBart's for HbH disease, HbF for β-thalassemia) may provide valuable information for screening.

Plasma fibronectin in patients undergoing major surgery

International Nuclear Information System (INIS)

Sallam, M.H.M.

2003-01-01

Plasma fibronectin in patients undergoing major surgery had been determined before and after operation. The study was done on 15 patients and 15 normal healthy individuals. The study revealed that patients subjected to major operation, their fibronectin level was normal before operation followed by reduction one day post-operation. After one week, fibronectin level raised again nearly to the pre-operations levels. The probable mechanisms of fibronectin in healing processes were discussed. Fibronectin (FN) is a family of structurally and immunologically related high molecular weight glycoproteins that are present in many cell surfaces, in extracellular fluids, in connective tissues and in most membranes. Interaction with certain discrete extracellular substances, such as a glucosaminoglycans (e.g. heparin), fibrin and collagen and with cell surface structure seem to account for many of its biological activities, among which are regulation of adhesion, spreading and locomotion (Mosesson and amrani, 1980). The concentration of Fn in human plasma decreases after extensive destruction such as that occurs in major surgery, burns or other trauma. This decrease has been generally though to be due to increased consumption of soluble plasma Fn in opsonization of particulate and soluble debris from circulation by the reticuloendothelial (RE) system. Fn rapidly appears in injury areas, in experimentally induced blisters, wounded and epithelium tissues (Petersen et al., 1985). Fn accumulates at times of increased vascular permeability and it is produced by cell of blood vessels in response to injury

Alloimmunization and autoimmunization in transfusion dependent thalassemia major patients: Study on 319 patients

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Hari Krishan Dhawan

2014-01-01

Full Text Available Background: The development of anti-red blood cell antibodies (both allo-and autoantibodies remains a major problem in thalassemia major patients. We studied the frequency of red blood cell (RBC alloimmunization and autoimmunization among thalassemia patients who received regular transfusions at our center and analyzed the factors, which may be responsible for development of these antibodies. Materials and Methods: The study was carried out on 319 multiply transfused patients with β-thalassemia major registered with thalassemia clinic at our institute. Clinical and transfusion records of all the patients were examined for age of patients, age at initiation of transfusion therapy, total number of blood units transfused, transfusion interval, status of splenectomy or other interventions. Alloantibody screening and identification was done using three cell and 11 cell panel (Diapanel, Bio-rad, Switzerland respectively. To detect autoantibodies, autocontrol was carried out using polyspecific coombs (IgG + C3d gel cards. Results: Eighteen patients out of total 319 patients (5.64% developed alloantibodies and 90 (28.2% developed autoantibodies. Nine out of 18 patients with alloantibodies also had autoantibodies. Age at first transfusion was significantly higher in alloimmunized than non-immunized patients (P = 0.042. Out of 23 alloantibodies, 52.17% belonged to Rh blood group system (Anti-E = 17%, Anti D = 13%, Anti-C = 13%, Anti-C w = 9%, 35% belonged to Kell blood group system, 9% of Kidd and 4% of Xg blood group system. Conclusion: Alloimmunization was detected in 5.64% of multitransfused thalassemia patients. Rh and Kell blood group system antibodies accounted for more than 80% of alloantibodies. This study re-emphasizes the need for RBC antigen typing before first transfusion and issue of antigen matched blood (at least for Rh and Kell antigen. Early institution of transfusion therapy after diagnosis is another means of decreasing

ASSOCIATION OF HFE GENE MUTATION IN THALASSEMIA MAJOR PATIENTS

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Amit Kumar Tiwari

2016-11-01

Full Text Available BACKGROUND Thalassemia major patients are dependent on frequent blood transfusion and consequently develop iron overload. HFE gene mutations (C282Y, H63D and S65C in hereditary haemochromatosis has been shown to be associated with iron overload. The study aims at finding the association of HFE gene mutations in β-thalassemia major patients. MATERIALS AND METHODS A descriptive observational pilot study was conducted including fifty diagnosed -thalassemia major cases. DNA analysis by PCR-RFLP method for HFE gene mutations was performed. RESULTS Only H63D mutation (out of three HFE gene mutations was detected in 8 out of 50 cases. Observed frequency of H63D mutation was 16%. While frequency of C282Y and S65C were 0% each. CONCLUSION The frequency of HFE mutation in -thalassemia major is not very common.

Pharmacological prevention of suicide in patients with major mood disorders.

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Rihmer, Zoltan; Gonda, Xenia

2013-12-01

The risk of self-destructive behavior in mood disorders is an inherent phenomenon and suicidal behavior in patients with unipolar or bipolar major mood disorders strongly relates to the presence and severity of depressive episodes. Consequently, early recognition, and successful acute and long-term treatment of depressive disorders is essential for suicide prevention in such patients. Large-scale, retrospective and prospective naturalistic long-term clinical studies, including severely ill, frequently suicidal depressives show that appropriate pharmacotherapy markedly reduces suicide morbidity and mortality even in this high-risk population. Supplementary psycho-social interventions further improve the effect. The slightly elevated (but in absolute sense quite low) risk of suicidal behavior among patients taking antidepressants compared to those taking placebo in randomized controlled antidepressant trials on unipolar major depression might be the consequence of the depression-worsening potential of antidepressant monotherapy in subthreshold and mixed bipolar depressed patients included in these trials and falsely diagnosed as suffering from unipolar major depression. Concurrent depression-focused psychotherapies increase the effectiveness of pharmacotherapy and this way contribute to suicide prevention for patients with mood disorders. Copyright © 2012 Elsevier Ltd. All rights reserved.

Does patient-provider gender concordance affect mental health care received by primary care patients with major depression?

Science.gov (United States)

Chan, Kitty S; Bird, Chloe E; Weiss, Robert; Duan, Naihua; Meredith, Lisa S; Sherbourne, Cathy D

2006-01-01

We sought to determine whether patient-provider gender concordance influences the detection and care of depression and comorbid anxiety and substance use in patients with major depression Cross-sectional analyses of baseline patient survey data linked with provider data were performed. Data based on routine primary care visits in clinics from a variety of health systems serving diverse patient populations across the United States. Participants all had major depression. Depression care was examined in the Quality Improvement for Depression (QID) Collaboration sample (n patients = 1,428, n providers = 389). In a subanalysis of data solely from 714 patients and 157 providers from Partners-In-Care, one of the projects participating in QID, we also examined detection of anxiety disorder and alcohol or drug problems. Rates of detection and care of mental health problems in primary care were low even among patients with major depression. Except for anxiety counseling in female patients, patient-provider gender concordance did not improve care as hypothesized. However, female providers were more likely to counsel on anxiety and less likely to counsel on alcohol or drug use than male providers. Female patients were less likely to be counseled on alcohol or drug use compared with male patients. Detection and care of mental health and substance use problems for patients with major depression is not influenced by patient-provider gender concordance. However, depressed female patients may have greater unmet needs for alcohol and drug use counseling than their male counterparts.

Association between the epidermal growth factor gene and intelligence in major depression patients.

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Tian, Wen-min; Zhang, Ke-ran; Zhang, Juan; Shen, Yan; Xu, Qi

2010-06-01

To study the association between the epidermal growth factor (EGF) gene and intelligence in patients with major depression. Intelligence measurement using Wechsler Adult Intelligence Scale (WAIS) was performed on 120 unrelated patients with major depression and 46 control subjects. Blood was collected from all subjects for extraction of genomic DNA. Four single nucleotide polymorphisms (SNPs) in the EGF gene were genotyped using polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI- TOF-MS). Mean scores of both score lang and score task, two subtests in WAIS, differed significantly between major depression patients and controls (Pintelligence in patients with major depression. Genetic variation in the EGF gene may increase the susceptibility of major depression.

[Hemoglobinopathies--clinical symptoms and diagnosis of thalassemia and abnormal hemoglobins].

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Herklotz, R; Risch, L; Huber, A R

2006-01-01

Haemoglobinopathies constitute entities that are generated by either an abnormal haemoglobin or thalassaemias. While abnormal haemoglobins are caused by a qualitative structural abnormality of the haemoglobin molecule, thalassaemias result by diminished synthesis of the globin chain. Due to increased immigration from Asia, Africa and the Mediterranean to Northern Europe, haemoglobin S, haemoglobin C, haemoglobin E are also encountered commonly in Switzerland, while other abnormal haemoglobins are rare, yet can cause clinically relevant symptoms. This include haemolysis, polyglobulia, cyanosis or a combination thereof Thalassaemia-syndroms constitute with two million affected individuals to the most prelevant monogenetic diseases worldwide. Due to migration into Switzerland, they are also found quite commonly among our patients with 10-15 per cent of all hypochromic, microcytic, anemia second only to iron deficiency. Importantly, thalassaemias and haemoglobinopathies can occur concomitantly sometimes even with a normal haemoglobin variant. This results in wide-spread presentations, making diagnosis and clinical judgement difficult. We describe in this article not only physiological mechanisms and clinical presentation but also propose a step-wise diagnostic algorithm including selective use of molecular biology methods.

Liver disease in adult transfusion-dependent beta-thalassaemic patients: investigating the role of iron overload and chronic HCV infection.

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Kountouras, Dimitrios; Tsagarakis, Nikolaos J; Fatourou, Evangelia; Dalagiorgos, Efthimios; Chrysanthos, Nikolaos; Berdoussi, Helen; Vgontza, Niki; Karagiorga, Markissia; Lagiandreou, Athanasios; Kaligeros, Konstantinos; Voskaridou, Ersi; Roussou, Paraskevi; Diamanti-Kandarakis, Evanthia; Koskinas, John

2013-03-01

Iron overload and hepatitis-C virus (HCV) infection, have been implicated in the evolution of liver disease, in patients with transfusion-dependent beta-thalassaemia major (BTM). However, the impact of these factors in late stages of liver disease in adults with BTM, has not been extensively studied. To investigate serum indices of iron overload, HCV infection and liver disease, in a cohort of 211 adult Greek patients with BTM, in relation with the findings from liver biopsies. In this cross-sectional study, 211 patients with BTM were enrolled and studied, in relation with HCV infection, ferritin, transaminases, chelation treatment and antiviral treatment. Based on 109 patients biopsied, we correlated liver fibrosis, haemosiderosis and inflammation, with serum indices and HCV status Among all patients, 74.4% were anti-HCV positive (HCV+). Ferritin was positively correlated with transaminases and negatively correlated with age, while it was not significantly different among HCV+ and HCV- patients. Among the HCV+ patients, 55.4% reported antiviral treatment, while genotype 1 predominated. In a subfraction of 109 patients, in which liver biopsy was performed, 89% were HCV+ and 11% HCV-. Fibrosis was significantly correlated with age (P = 0.046), AST (P = 0.004), ALT (P = 0.044) and inflammation (P overload may be the critical determinant, since fibrosis is related to the minimal haemosiderosis, independently of HCV history. © 2012 John Wiley & Sons A/S.

Addressing adherence to treatment: a longstanding concern. The health care professional

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Farrukh Shah

2014-12-01

Full Text Available Today a patient born with thalassaemia major can expect to have a near normal life expectancy and remain free of complications of iron overload with good monitoring and excellent transfusion and chelation regimes. Unfortunately patients still develop complications as a consequence of iron overload including endocrinopathies and cardiac failure. The main reason behind this failure of effective treatment is inadequate treatment. This can be due to either clinician related factors, patient related factors or lack of adequate provision of medicines and services. In this short paper I will highlight where the challenges lie with regards adherence to treatment and suggest approaches to manage this.

MODULATING EFFECT OF THE −158 GΓ (C→T XMN-1 POLYMORPHISM IN INDIAN SICKLE CELL PATIENTS

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Sanjay Pandey

2012-01-01

Full Text Available Xmn-1 polymorphism is a known factor, which increases fetal haemoglobin production. Among the inherited disorders of blood, thalassaemia and SCD constitutes a major bulk of genetic diseases in India.  Our aim was to verify the role of the Xmn I polymorphism as a modulating factor in sickle cell patients and frequency of the polymorphism in Indian sickle cell patients. Subjects were 60 sickles homozygous and 75 sickle beta thalassemia patients. 5 ml blood   samples collected from patients. Screening of sickle patients done by HPLC. An automated cell analyzer SYSMEX (K-4500 Model used to analyze the CBC of patients.Xmn1 polymorphism analysis done by PCR-RFLP and Statistical analysis was performed on GraphPad static’s software. t test applied to compare the means amongst group. Among the sickle homozygous 27 were   heterozygous (+/- and 19 were   homozygous (+/+ while 30 were heterozygous and 24 were homozygous in sickle β-thalassemia patients. Extremely significant differences (p-value <0.001 of hematological parameters seen among patient with xmn-1 carrier and without the xmn-1 carrier. In our cases the clinical symptom less frequent and higher HbF level with Xmn-1 carriers. Presence of Xmn-1 polymorphism in sickle patients with higher HbF that improve phenotypic presentation in the sickle cell patients. We conclude that the phenotype of Indian sickle cell patients influenced by Xmn-1 polymorphism.

Renal tubular dysfunction in pediatric patients with beta-thalassemia major

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Ali Ahmadzadeh

2011-01-01

Full Text Available To evaluate the prevalence of renal tubular dysfunction in children with β-thalassemia (β-T major, we studied the glomerular and tubular function in 140 children with β-T major and compared them to a healthy control group at our center from May 2007 to April 2008. Fresh first morning samples were collected from each patient and analyzed for sodium, potassium, calcium (Ca, protein, uric acid (UA, creatinine (Cr, urine osmolality and urinary N-acetyl-β-D-glucosaminidase (UNAG activity. Blood samples were also collected for complete blood count, blood urea nitrogen (BUN, fasting blood sugar, serum creatinine (SCr, electrolytes, and ferritin before transfusion. Among the study patients, 72 were males, and the mean age was 11.5 (ranging 7-16 years. SCr levels were all within normal limits and all of them had normal glomerular filtration rate (GFR. The mean UNAG was 17.8 IU/L in the study patients (normal 0.15-11.5 IU/L and 3.2 IU/L in the control group (P 0.21 (P = 0.006. Nine (6.4% thalassemic patients with a mean age of 12 years had proteinuria (Upr/UCr > 0.2. Sixty-nine (49.3% out of the 140 patients and 45 (65.2% of the patients having UNAG had uricosuria also (UUA/UCr > 0.26. Ten (7% patients had microscopic hematuria and 10 (7% patients with a mean age of 13.5 years had glucosuria or diabetes mellitus. We conclude that tubular dysfunction is a relative common complication of the β-T major; UNAG and its index are the best to detect renal tubular dysfunction in these patients. Currently, periodic measurement of UCa/UCr and UUA/UCr ratios as well as urinalysis are recommended.

Laboratory diagnosis of the rare anaemias: external quality assessment benefits patient care

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Barbara De La Salle

2013-03-01

Full Text Available Since its introduction in the 1960s, external quality assessment has developed to become an essential component of the quality management system of the diagnostic laboratory. External quality assessment provides a long term, retrospective view of laboratory performance, demonstrating the competence of the laboratory to others. The ENERCA project (the European Network for Rare and Congenital Anaemias has established a list of core laboratory tests that are used in the diagnosis of rare and congenital anaemias, which has been used as the basis for questionnaires to laboratories, to establish the use and quality assurance of diagnostic testing in the congenital and rare anaemias, and to European EQA providers for services in this key area. In general, the provision of EQA for rare and congenital anaemias is widely variable with little provision for the very rare disorders. For the more common congenital anaemias, such as the haemoglobinopathies and thalassaemias, provision is better but there is variation in aspects of the scheme design, especially the frequency of distribution. Where laboratories did not take part in EQA for individual tests, or there was no EQA available, a desire to participate was expressed in 66% (102/154 of cases. The provision of external quality assessment (EQA services for rare disorders is a challenge. For many of these conditions, the number of patients in any one member state is very small with only a few laboratories providing diagnostic testing. In these cases, the development of pan-European or cross-border EQA may be the only means by which standardisation of methods and results can be achieved. An EQA survey of 243 laboratories for performance in Hb A2 quantification showed encouraging results in that there was a clear differentiation in the results from a beta Thalassaemia carrier and an individual with no evidence of Thalassaemia; however, a bias was observed between different methods of measurement.

Impaired intuition in patients with major depressive disorder.

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Remmers, Carina; Topolinski, Sascha; Dietrich, Detlef E; Michalak, Johannes

2015-06-01

In daily life, many decisions of minor and major importance have to be made. Thereby, intuitive judgments serve as useful guides and help us to adapt to our environment. People with major depressive disorder (MDD) often have difficulties to come to decisions. Is their intuition impaired? Since this question has not been addressed until now, the present study explored intuition in MDD. Depressed patients (n = 29) and healthy control participants (n = 27) completed the Judgment of Semantic Coherence Task, a well-established paradigm used in basic cognitive research to measure intuition. Furthermore, participants' severity of depressive symptoms (BDI-II), negative affect (PANAS), and rumination (RSQ) were assessed. All participants were interviewed with the SCID. Depressed patients showed impaired intuition compared to healthy control participants. In the depressed sample, negative affect accounts for the association between rumination and impaired intuition. Results further reveal that negative affect overall mediates the depression-intuition relationship. Patients with diminished ability to concentrate or indecisiveness had lower intuition indices compared to patients who did not fulfil this diagnostic criterion of MDD. The study introduces the phenomenon of intuition into depression research. Additionally, these results extent findings from basic research showing that induced negative mood as well difficulties to down-regulate negative affect impair intuitive coherence judgments. Current results indicate that the negative affectivity of patients is the crucial mediator in the association between depression and impaired intuition. Limitations of the study as well as the potential etiological role of intuition in MDD are discussed. The finding that intuition is impaired in depressed patients extends our knowledge as to the cognitive profile of patients with MDD. Patients who suffer from indecisiveness have lower intuition indices compared to patients who do not

Identification of major cardiovascular events in patients with diabetes using primary care data.

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Pouwels, Koen Bernardus; Voorham, Jaco; Hak, Eelko; Denig, Petra

2016-04-02

Routine primary care data are increasingly being used for evaluation and research purposes but there are concerns about the completeness and accuracy of diagnoses and events captured in such databases. We evaluated how well patients with major cardiovascular disease (CVD) can be identified using primary care morbidity data and drug prescriptions. The study was conducted using data from 17,230 diabetes patients of the GIANTT database and Dutch Hospital Data register. To estimate the accuracy of the different measures, we analyzed the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) relative to hospitalizations and/or records with a diagnosis indicating major CVD, including ischaemic heart diseases and cerebrovascular events. Using primary care morbidity data, 43% of major CVD hospitalizations could be identified. Adding drug prescriptions to the search increased the sensitivity up to 94%. A proxy of at least one prescription of either a platelet aggregation inhibitor, vitamin k antagonist or nitrate could identify 85% of patients with a history of major CVD recorded in primary care, with an NPV of 97%. Using the same proxy, 57% of incident major CVD recorded in primary or hospital care could be identified, with an NPV of 99%. A substantial proportion of major CVD hospitalizations was not recorded in primary care morbidity data. Drug prescriptions can be used in addition to diagnosis codes to identify more patients with major CVD, and also to identify patients without a history of major CVD.

Nitroblue tetrazolium test in patients with beta-thalassemia major

International Nuclear Information System (INIS)

Ghaffari, J.; Vahidshahi, K.; Kosaryan, M.; Karami, H.; Mahdavi, M.; Parvinnejad, N.

2008-01-01

Objective to assess the neutrophil function in thalassemia major (TM)patients and compare it with the control group and to recognize its relevantfactors. This was a retrospective cohort study, which was carried out fromOctober 2007 to February 2008 in the Thalassemia research Center in Boo AliSina Hospital in Sari, Mazandaran, North of Iran. The study populationconsisted of TM patients in Boo Ali Sina Teaching Hospital. The method ofsampling in the case group was systematic and it was target based in thecontrol group. The sample size determined was based on previous studies.Thalassemia major was diagnosed based on hemoglobin electrophoresis (casegroup). The control group was their brothers and sisters, who had +-5 yearsof age difference and were of the same gender as the patients. Datacollection was based on interview, investigating demographic characteristicsand also obtaining medical information from medical records of the patients.The neutrophil function was by performing nitroblue tetrazolium (NBT)reduction test. The test was carried out on both groups and the data wereanalyzed by software using SPSS version 13.0. In this study, 39 patients and39 healthy controls were compared. The average age of the patients was21.6+-5.3 years and it was 22.4+-5.1 years in healthy controls (p=0.7). Therewas a significant correlation between the test's results and the patients age(p=0.008). The rate of impaired NBT results in the patients was 36%, while itwas 10% in controls, which were significantly different. The neutrophilactivity based on NBT test was 89.9+-11.6% in the case group and 93.7+-2.51%in the control group (p=0.025). This study indicates that neutrophil activityin thalassemia patients was significantly lower, compared to the normalcontrol group, especially in young patients. Based on the results, evaluationof neutrophil function and pyogenic infections in TM patients seemsnecessary. (author)

Nitric Oxide-Related Biological Pathways in Patients with Major Depression.

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Andreas Baranyi

Full Text Available Major depression is a well-known risk factor for cardiovascular diseases and increased mortality following myocardial infarction. However, biomarkers of depression and increased cardiovascular risk are still missing. The aim of this prospective study was to evaluate, whether nitric-oxide (NO related factors for endothelial dysfunction, such as global arginine bioavailability, arginase activity, L-arginine/ADMA ratio and the arginine metabolites asymmetric dimethylarginine (ADMA and symmetric dimethylarginine (SDMA might be biomarkers for depression-induced cardiovascular risk.In 71 in-patients with major depression and 48 healthy controls the Global Arginine Bioavailability Ratio (GABR, arginase activity (arginine/ornithine ratio, the L-arginine/ADMA ratio, ADMA, and SDMA were determined by high-pressure liquid chromatography. Psychiatric and laboratory assessments were obtained at baseline at the time of in-patient admittance and at the time of hospital discharge.The ADMA concentrations in patients with major depression were significantly elevated and the SDMA concentrations were significantly decreased in comparison with the healthy controls. Even after a first improvement of depression, ADMA and SDMA levels remained nearly unchanged. In addition, after a first improvement of depression at the time of hospital discharge, a significant decrease in arginase activity, an increased L-arginine/ADMA ratio and a trend for increased global arginine bioavailability were observed.Our study results are evidence that in patients with major depression ADMA and SDMA might be biomarkers to indicate an increased cardiovascular threat due to depression-triggered NO reduction. GABR, the L-arginine/ADMA ratio and arginase activity might be indicators of therapy success and increased NO production after remission.

Effect ALPHA Globalin Gene Deletion and GAMMA Globin Gene -158 (C/T) Polymorphism in BETA- Thalassaemic Patients

International Nuclear Information System (INIS)

EL Serafi, T.I.; Ismail, E.F.; Mahmoud, M.A.; Mohamed, M.A.; Ghattas, M.H.; Badran, D.I.; El Serafi, I.T.; Mohamed, H.S.

2008-01-01

The beta-thalassemias (β- thalassemias) are among the most common autosomal recessive disorders. They have a remarkably high frequency in the Mediterranean region and represent one of the most common genetic diseases in Egypt. In this study, the spectrum of P- thalassemia mutations and genotype-to-phenotype correlations were defined in 32 β- thalassaemic patients (β- thalassemias major and intermedia) with varying disease severity in two cities of the Suez Canal region. Ten different mutations were identified and the most frequent ones were: Isi-6 (T-C) (37.5%), IVSI-110 (G-A) (34.4%) and both IVSI-1 (G-A), IVSII-745 (C-G) and -102 (C-G) (12.5% each). There was a wide spectrum of phenotypic severity in all patients. We studied the Xmnl polymorphism (C/T) in γ- globin gene position -158 of P- thalassemia as a modulating factor of the disease severity. Presence of the polymorphism was found in two patients and this was not sufficient to explain the diversity of the phenotype encountered. Co-inheritance of alpha thalassaemia as a modulating factor was not evident in our patients. In conclusion, we have been unable to find a molecular basis for the benign clinical course in all our patients. Other genetic or acquired factors must be hypothesized which ameliorate the clinical condition.

Psychiatric comorbidities in patients with major depressive disorder

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Thaipisuttikul P

2014-11-01

Full Text Available Papan Thaipisuttikul, Pichai Ittasakul, Punjaporn Waleeprakhon, Pattarabhorn Wisajun, Sudawan Jullagate Department of Psychiatry, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Background: Psychiatric comorbidities are common in major depressive disorder (MDD. They may worsen outcome and cause economic burden. The primary objective was to examine the prevalence of psychiatric comorbidities in MDD. The secondary objectives were to compare the presence of comorbidities between currently active and past MDD, and between patients with and without suicidal risk.Methods: This was a cross-sectional study. A total of 250 patients with lifetime MDD and age ≥18 years were enrolled. The Mini International Neuropsychiatric Interview (MINI, Thai version, was used to confirm MDD diagnosis and classify comorbidities. MDD diagnosis was confirmed in 190, and 60 patients were excluded due to diagnosis of bipolar disorder.Results: Of the 190 MDD patients, 25.8% had current MDD and 74.2% had past MDD. Eighty percent were women. The mean age at enrollment was 50 years, and at MDD onset was 41 years. Most patients were married (53.2%, employed (54.8%, and had ≥12 years of education (66.9%. There were 67 patients (35.3% with one or more psychiatric comorbidities. Comorbidities included dysthymia (19.5%, any anxiety disorders (21.1% (panic disorder [6.8%], agoraphobia [5.8%], social phobia [3.7%], obsessive–compulsive disorder [OCD] [4.7%], generalized anxiety disorder [5.3%], and post-traumatic stress disorder [4.2%], alcohol dependence (0.5%, psychotic disorder (1.6%, antisocial personality (1.1%, and eating disorders (0%. Compared with past MDD, the current MDD group had significantly higher OCD (P<0.001, psychotic disorder (P=0.048, past panic disorder (P=0.017, and suicidal risk (P<0.001. Suicidal risk was found in 32.1% of patients. Patients with suicidal risk had more comorbid anxiety disorder of any type (P=0.019 and

The portfolio method as management support for patients with major depression.

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Nunstedt, Håkan; Nilsson, Kerstin; Skärsäter, Ingela

2014-06-01

To describe how patients with major depression in psychiatric outpatient care use the portfolio method and whether the method helps the patients to understand their depression. Major depressive disorder is an increasing problem in society. Learning about one's depression has been demonstrated to be important for recovery. If the goal is better understanding and management of depression, learning must proceed on the patient's own terms, based on the patient's previous understanding of their depression. Learning must be aligned with patient needs if it is to result in meaningful and useful understanding. Each patient's portfolio consisted of a binder. Inside the binder, there was a register with predetermined flaps and questions. The patients were asked to work with the questions in the sections that built the content in the portfolio. Individual interviews with patients (n = 5) suffering from major depression according to Diagnostic and Statistical Manual of Mental Disorders - Fourth Edition (DSM-IV) (American Psychiatric Association 1994) were repeatedly conducted between April 2008 and August 2009 in two psychiatric outpatient clinics in western Sweden. Data were analysed using latent content analysis. The results showed that the portfolio was used by patients as a management strategy for processing and analysis of their situation and that a portfolio's structure affects its usability. The patients use the portfolio for reflection on and confirmation of their progress, to create structure in their situation, as a management strategy for remembering situations and providing reminders of upcoming activities. Using a clearly structured care portfolio can enable participation and patient learning and help patients understand their depression. The portfolio method could provide a tool in psychiatric nursing that may facilitate patient understanding and increase self-efficacy. © 2013 John Wiley & Sons Ltd.

Human parvovirus B19 in patients with beta thalassemia major from Tehran, Iran.

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Arabzadeh, Seyed Ali Mohammad; Alizadeh, Farideh; Tavakoli, Ahmad; Mollaei, Hamidreza; Bokharaei-Salim, Farah; Karimi, Gharib; Farahmand, Mohammad; Mortazavi, Helya Sadat; Monavari, Seyed Hamidreza

2017-03-01

Due to the tropism of human parvovirus B19 to erythroid progenitor cells, infection in patients with an underlying hemolytic disorder such as beta-thalassemia major leads to suppression of erythrocyte formation, referred to as transient aplasia crisis (TAC), which may be life-threatening. We investigated the prevalence of parvovirus B19 among patients with beta thalassemia major attending the Zafar Adult Thalassemia Clinic in Tehran, Iran. This cross-sectional study was performed to determine the presence of parvovirus B19 DNA in blood samples and parvovirus B19 genotypes in plasma samples of patients with thalassemia major. The population consisted of 150 patients with beta-thalassemia major who attended the Zafar clinic in Tehran. Specimens were studied using a real-time polymerase chain reaction assay. The prevalence of parvovirus B19 in our study population was 4%. Of 150 patients with thalassemia, six (4%) were positive for B19 DNA. There was no significant correlation between blood transfusion frequency and B19 DNA positivity. Finally, phylogenetic analysis of human parvovirus B19 revealed genotype I in these six patients. In this study, acute B19 infections were detected in patients with beta thalassemia major. Screening of such high-risk groups can considerably reduce the incidence and prevalence of B19 infection; thus, screening is required for epidemiologic surveillance and disease-prevention measures.

Neural mechanisms of reinforcement learning in unmedicated patients with major depressive disorder.

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Rothkirch, Marcus; Tonn, Jonas; Köhler, Stephan; Sterzer, Philipp

2017-04-01

According to current concepts, major depressive disorder is strongly related to dysfunctional neural processing of motivational information, entailing impairments in reinforcement learning. While computational modelling can reveal the precise nature of neural learning signals, it has not been used to study learning-related neural dysfunctions in unmedicated patients with major depressive disorder so far. We thus aimed at comparing the neural coding of reward and punishment prediction errors, representing indicators of neural learning-related processes, between unmedicated patients with major depressive disorder and healthy participants. To this end, a group of unmedicated patients with major depressive disorder (n = 28) and a group of age- and sex-matched healthy control participants (n = 30) completed an instrumental learning task involving monetary gains and losses during functional magnetic resonance imaging. The two groups did not differ in their learning performance. Patients and control participants showed the same level of prediction error-related activity in the ventral striatum and the anterior insula. In contrast, neural coding of reward prediction errors in the medial orbitofrontal cortex was reduced in patients. Moreover, neural reward prediction error signals in the medial orbitofrontal cortex and ventral striatum showed negative correlations with anhedonia severity. Using a standard instrumental learning paradigm we found no evidence for an overall impairment of reinforcement learning in medication-free patients with major depressive disorder. Importantly, however, the attenuated neural coding of reward in the medial orbitofrontal cortex and the relation between anhedonia and reduced reward prediction error-signalling in the medial orbitofrontal cortex and ventral striatum likely reflect an impairment in experiencing pleasure from rewarding events as a key mechanism of anhedonia in major depressive disorder. © The Author (2017). Published by Oxford

Bone metabolism and mineral density in patients with beta-thalassemia major

International Nuclear Information System (INIS)

Dundar, U.; Kupesiz, A.; Yesilipeck, A.; Ozdem, S.; Gultekin, M.; Gilgil, E.; Tuncer, T.

2007-01-01

To evaluate bone metabolism in patients with beta-thalassemia major and to determine the factors associated with the development of osteoporosis. We studied 25 patients with thalassemia major with a mean age of 18.4 years (rang 5-31), age and gender matched 24 healthy controls who were attending the outpatient physical medicine and rehabilitation clinic of Akdeniz University Hospital between January 2004 and March 2004 in Turkey. Bone mineral density (BMD) of lumbar spine (L-1-L4) and proximal femur were determined using dual x-ray absorptiometry (DXA). Venous blood samples were obtained for determination of blood cell count and markers of bone formation and resorption. The BMD values, both at lumbar and femoral neck levels were significantly lower in patients compared to controls. Serum N-telopeptide level was slightly higher, whereas osteocalcin was slightly lower in patients, however, the values were not statistically significant. Plasma levels of insulin like growth factor-1 (IGF-I) and insulin like growth factor for binding protein-3 (IGFBP-3) were significantly lower in patients. Also, serum levels of estradiol and progesterone in females, luteinizing, hormone and follicle-stimulating hormone in both genders were significantly lower in patients. Serum levels of free testosterone and total testosterone were lower in patients, but not statistically significant. Patients also had significantly higher serum phosphorous levels and lower serum calcitonin levels compared to controls. The BMD is decreased in thalassemic patients. Growth retardation, growth hormone/IGF-I/IGFP-3 axis dysfunction, gonadal dysfunction and hypothalomo-pituitary-gonadal axis dysfunction may be responsible for the development of osteoporosis in the patients with beta-thalassemia major. (author)

Paracetamol, Ibuprofen, and Recurrent Major Cardiovascular and Major Bleeding Events in 19 120 Patients With Recent Ischemic Stroke.

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Gonzalez-Valcarcel, Jaime; Sissani, Leila; Labreuche, Julien; Bousser, Marie-Germaine; Chamorro, Angel; Fisher, Marc; Ford, Ian; Fox, Kim M; Hennerici, Michael G; Mattle, Heinrich P; Rothwell, Peter M; Steg, Philippe Gabriel; Vicaut, Eric; Amarenco, Pierre

2016-04-01

The presumed safety of paracetamol in high-cardiovascular risk patients has been questioned. We determined whether paracetamol or ibuprofen use is associated with major cardiovascular events (MACE) or major bleeding in 19 120 patients with recent ischemic stroke or transient ischemic attack of mainly atherothrombotic origin included in the Prevention of cerebrovascular and cardiovascular events of ischemic origin with terutroban in patients with a history of ischemic stroke or transient ischemic attack (PERFORM) trial. We performed 2 nested case-control analysis (2153 cases with MACE during trial follow-up and 4306 controls matched on Essen stroke risk score; 809 cases with major bleeding matched with 1616 controls) and a separate time-varying analysis. 12.3% were prescribed paracetamol and 2.5% ibuprofen. Median duration of treatment was 14 (interquartile range 5-145) days for paracetamol and 9 (5-30) days for ibuprofen. Paracetamol, but not ibuprofen, was associated with increased risk of MACE (odds ratio 1.21, 95% confidence interval [CI] 1.04-1.42) or a major bleeding (odds ratio 1.60, 95% CI 1.26-2.03), with no impact of daily dose and duration of paracetamol treatment. Time-varying analysis found an increased risk of MACE with both paracetamol (hazard ratio 1.22, 95% CI 1.05-1.43) and ibuprofen (hazard ratio 1.47, 95% CI 1.06-2.03) and of major bleeding with paracetamol (hazard ratio 1.95, 95% CI 1.45-2.62). There was a weak and inconsistent signal for association between paracetamol or ibuprofen and MACE or major bleeding, which may be related to either a genuine but modest effect of these drugs or to residual confounding. http://www.isrctn.com. Unique identifier: ISRCTN66157730. © 2016 American Heart Association, Inc.

CLINICAL APPLICATION OF RECOMBINANT ERYTHROPOIETIN IN BETA-THALASSAEMIA INTERMEDIA.

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Asadov, Ch; Alimirzoyeva, Z; Hasanova, M; Mammadova, T; Shirinova, A

2016-06-01

Research objective is to study the efficacy of recombinant erythropoietin (epoetin alfa) as alternative method of treatment beta-thalassemia intermedia. Study involved 58 patients with beta-thalassemia intermedia (23 women and 35 men). In all observed patients was defined levels of hemoglobin (Hb), red blood cells (RBC), erythrocyte indexes (MCV, MCH, MCHC), hemoglobin fractions (HbA, HbA2, HbF), serum ferritin, serum erythropoietin before and after administrated rEPO. All patients received rEPO during 6 month at the dose - 10000 IU subcutaneously. The majority of patients - 39 (67%) had a good response to rEPO (increase in hemoglobin level more than 20 g/l); 16 patients (28%) had a mean response (increase in Hb 10 - 20 g/l); in 3 (5%) patients occurred poor response to rEPO therapy (increase in Hb intermedia patients there was a statistically significant change in the number of RBC, levels of HbF and sEPO. The evaluation of interdependence between the indices of the baseline sEPO and increased Hb values in patients after rEPO treatment revealed the presence of the reverse direct relationship (r=-0.67). Based on the results, it can be concluded that the use of rEPO in complex therapy of beta-thalassemia intermedia leads to increased levels of Hb and consequently reducing the need for blood transfusions, and accordingly expected to prevent severe complications of blood transfusion (alloimmunization, hypersplenism, iron overload, contamination transmissible infections) facilitating normal growth and development, and a better quality of life.

Functional Recovery in Major Depressive Disorder: Providing Early Optimal Treatment for the Individual Patient

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Katzman, Martin A; Habert, Jeffrey; McIntosh, Diane; MacQueen, Glenda M; Milev, Roumen V; McIntyre, Roger S; Blier, Pierre

2018-01-01

Abstract Major depressive disorder is an often chronic and recurring illness. Left untreated, major depressive disorder may result in progressive alterations in brain morphometry and circuit function. Recent findings, however, suggest that pharmacotherapy may halt and possibly reverse those effects. These findings, together with evidence that a delay in treatment is associated with poorer clinical outcomes, underscore the urgency of rapidly treating depression to full recovery. Early optimized treatment, using measurement-based care and customizing treatment to the individual patient, may afford the best possible outcomes for each patient. The aim of this article is to present recommendations for using a patient-centered approach to rapidly provide optimal pharmacological treatment to patients with major depressive disorder. Offering major depressive disorder treatment determined by individual patient characteristics (e.g., predominant symptoms, medical history, comorbidities), patient preferences and expectations, and, critically, their own definition of wellness provides the best opportunity for full functional recovery. PMID:29024974

Mental rotation evoked potentials P500 in patients with major depressive disorder

Institute of Scientific and Technical Information of China (English)

陈玖

2013-01-01

Objective To explore the difference on mental rotation ability between major depressive disorders and healthy subjects.Methods Twenty-three patients with major depressive disorders and 24 healthy subjects

Professionally responsible intrapartum management of patients with major mental disorders.

Science.gov (United States)

Babbitt, Kriste E; Bailey, Kala J; Coverdale, John H; Chervenak, Frank A; McCullough, Laurence B

2014-01-01

Pregnant women with major mental disorders present obstetricians with a range of clinical challenges, which are magnified when a psychotic or agitated patient presents in labor and there is limited time for decision making. This article provides the obstetrician with an algorithm to guide professionally responsible decision making with these patients. We searched for articles related to the intrapartum management of pregnant patients with major mental disorders, using 3 main search components: pregnancy, chronic mental illness, and ethics. No articles were found that addressed the clinical ethical challenges of decision making during the intrapartum period with these patients. We therefore developed an ethical framework with 4 components: the concept of the fetus as a patient; the presumption of decision-making capacity; the concept of assent; and beneficence-based clinical judgment. On the basis of this framework we propose an algorithm to guide professionally responsible decision making that asks 5 questions: (1) Does the patient have the capacity to consent to treatment?; (2) Is there time to attempt restoration of capacity?; (3) Is there an opportunity for substituted judgment?; (4) Is the patient accepting treatment?; (5) Is there an opportunity for active assent?; and (6) coerced clinical management as the least worst alternative. The algorithm is designed to support a deliberative, clinically comprehensive, preventive-ethics approach to guide obstetricians in decision making with this challenging population of patients. Copyright © 2014 Mosby, Inc. All rights reserved.

Fluid resuscitation for major burn patients with the TMMU protocol.

Science.gov (United States)

Luo, Gaoxing; Peng, Yizhi; Yuan, Zhiqiang; Cheng, Wenguang; Wu, Jun; Tang, Jin; Huang, Yuesheng; Fitzgerald, Mark

2009-12-01

Fluid resuscitation is one of the critical treatments for the major burn patient in the early phases after injury. We evaluated the practice of fluid resuscitation for severely burned patients with the Third Military Medical University (TMMU) protocol, which is most widely used in many regions of China. Patients with major burns (>30% total body surface area (TBSA)) presenting to Southwest Hospital, Third Military Medical University, between January 2005 and October 2007, were included in this study. Fluid resuscitation was initiated by the TMMU protocol. A total of 71 patients were (46 adults and 25 children) included in this study. All patients survived the first 48 h after injury smoothly and none developed abdominal compartment syndrome or other recognised complications associated with fluid resuscitation. The average quantity of fluid infused was 3.3-61.33% more than that calculated based on the TMMU protocol in both adult and paediatric groups. The average urine output during the first 24h after injury was about 1.2 ml per kg body weight per hour in the two groups, but reached 1.2 ml and 1.7 ml during the second 24h in adult and pediatric groups, respectively. This study indicates that the TMMU protocol for fluid resuscitation is a feasible option for burn patients. Individualised resuscitation - guided by the physiological response to fluid administration - is still important as in other protocols.

Insular and Hippocampal Gray Matter Volume Reductions in Patients with Major Depressive Disorder

Science.gov (United States)

Kugel, Harald; Krug, Axel; Schöning, Sonja; Ohrmann, Patricia; Uhlmann, Christina; Postert, Christian; Suslow, Thomas; Heindel, Walter; Arolt, Volker; Kircher, Tilo; Dannlowski, Udo

2014-01-01

Background Major depressive disorder is a serious psychiatric illness with a highly variable and heterogeneous clinical course. Due to the lack of consistent data from previous studies, the study of morphometric changes in major depressive disorder is still a major point of research requiring additional studies. The aim of the study presented here was to characterize and quantify regional gray matter abnormalities in a large sample of clinically well-characterized patients with major depressive disorder. Methods For this study one-hundred thirty two patients with major depressive disorder and 132 age- and gender-matched healthy control participants were included, 35 with their first episode and 97 with recurrent depression. To analyse gray matter abnormalities, voxel-based morphometry (VBM8) was employed on T1 weighted MRI data. We performed whole-brain analyses as well as a region-of-interest approach on the hippocampal formation, anterior cingulate cortex and amygdala, correlating the number of depressive episodes. Results Compared to healthy control persons, patients showed a strong gray-matter reduction in the right anterior insula. In addition, region-of-interest analyses revealed significant gray-matter reductions in the hippocampal formation. The observed alterations were more severe in patients with recurrent depressive episodes than in patients with a first episode. The number of depressive episodes was negatively correlated with gray-matter volume in the right hippocampus and right amygdala. Conclusions The anterior insula gray matter structure appears to be strongly affected in major depressive disorder and might play an important role in the neurobiology of depression. The hippocampal and amygdala volume loss cumulating with the number of episodes might be explained either by repeated neurotoxic stress or alternatively by higher relapse rates in patients showing hippocampal atrophy. PMID:25051163

Higher incidence of major complications after splenic embolization for blunt splenic injuries in elderly patients.

Science.gov (United States)

Wu, Shih-Chi; Fu, Chih-Yuan; Chen, Ray-Jade; Chen, Yung-Fang; Wang, Yu-Chun; Chung, Ping-Kuei; Yu, Shu-Fen; Tung, Cheng-Cheng; Lee, Kun-Hua

2011-02-01

Nonoperative management (NOM) of blunt splenic injuries has been widely accepted, and the application of splenic artery embolization (SAE) has become an effective adjunct to NOM. However, complications do occur after SAE. In this study, we assess the factors leading to the major complications associated with SAE. Focusing on the major complications after SAE, we retrospectively studied patients who received SAE and were admitted to 2 major referral trauma centers under the same established algorithm for management of blunt splenic injuries. The demographics, angiographic findings, and factors for major complications after SAE were examined. Major complications were considered to be direct adverse effects arising from SAE that were potentially fatal or were capable of causing disability. There were a total of 261 patients with blunt splenic injuries in this study. Of the 261 patients, 53 underwent SAE, 11 (21%) of whom were noted to have 12 major complications: 8 cases of postprocedural bleeding, 2 cases of total infarction, 1 case of splenic abscess, and 1 case of splenic atrophy. Patients older than 65 years were more susceptible to major complications after SAE. Splenic artery embolization is considered an effective adjunct to NOM in patients with blunt splenic injuries. However, risks of major complications do exist, and being elderly is, in part, associated with a higher major complication incidence. Copyright © 2011 Elsevier Inc. All rights reserved.

Epidemiology and clinical impact of major comorbidities in patients with COPD

Science.gov (United States)

Smith, Miranda Caroline; Wrobel, Jeremy P

2014-01-01

Comorbidities are frequent in chronic obstructive pulmonary disease (COPD) and significantly impact on patients’ quality of life, exacerbation frequency, and survival. There is increasing evidence that certain diseases occur in greater frequency amongst patients with COPD than in the general population, and that these comorbidities significantly impact on patient outcomes. Although the mechanisms are yet to be defined, many comorbidities likely result from the chronic inflammatory state that is present in COPD. Common problems in the clinical management of COPD include recognizing new comorbidities, determining the impact of comorbidities on patient symptoms, the concurrent treatment of COPD and comorbidities, and accurate prognostication. The majority of comorbidities in COPD should be treated according to usual practice, and specific COPD management is infrequently altered by the presence of comorbidities. Unfortunately, comorbidities are often under-recognized and under-treated. This review focuses on the epidemiology of ten major comorbidities in patients with COPD. Further, we emphasize the clinical impact upon prognosis and management considerations. This review will highlight the importance of comorbidity identification and management in the practice of caring for patients with COPD. PMID:25210449

Major trauma from suspected child abuse: a profile of the patient pathway.

Science.gov (United States)

Davies, Ffion C; Lecky, Fiona E; Fisher, Ross; Fragoso-Iiguez, Marisol; Coats, Tim J

2017-09-01

Networked organised systems of care for patients with major trauma now exist in many countries, designed around the needs of the majority of patients (90% adults). Non-accidental injury is a significant cause of paediatric major trauma and has a different injury and age profile from accidental injury (AI). This paper compares the prehospital and inhospital phases of the patient pathway for children with suspected abuse, with those accidentally injured. The paediatric database of the national trauma registry of England and Wales, Trauma Audit and Research Network, was interrogated from April 2012 (the launch of the major trauma networks) to June 2015, comparing the patient pathway for cases of suspected child abuse (SCA) with AI. In the study population of 7825 children, 7344 (94%) were classified as AI and 481 (6%) as SCA. SCA cases were younger (median 0.4 years vs 7 years for AI), had a higher Injury Severity Score (median 16vs9 for AI), and had nearly three times higher mortality (5.7%vs2.2% for AI). Other differences included presentation to hospital evenly throughout the day and year, arrival by non-ambulance means to hospital (74%) and delayed presentation to hospital from the time of injury (median 8 hours vs 1.8 hours for AI). Despite more severe injuries, these infants were less likely to receive key interventions in a timely manner. Only 20% arrived to a designated paediatric-capable major trauma centre. Secondary transfer to specialist care, if needed, took a median of 21.6 hours from injury(vs 13.8 hours for AI). These data show that children with major trauma that is inflicted rather than accidental follow a different pathway through the trauma system. The current model of major trauma care is not a good fit for the way in which child victims of suspected abuse present to healthcare. To achieve better care, awareness of this patient profile needs to increase, and trauma networks should adjust their conventional responses. © Article author

Parathyroid hormone in pediatric patients with β-thalassemia major ...

African Journals Online (AJOL)

The aim of the study is to estimate the level of serum parathyroid hormone and its relation to bone mineral density in transfusion dependent beta-thalassemia major children. Subjects and methods: We measured serum calcium, phosphorus and parathyroid hormone in a sample of pediatric patients with thalassemia, ...

Nursing research on a first aid model of double personnel for major burn patients.

Science.gov (United States)

Wu, Weiwei; Shi, Kai; Jin, Zhenghua; Liu, Shuang; Cai, Duo; Zhao, Jingchun; Chi, Cheng; Yu, Jiaao

2015-03-01

This study explored the effect of a first aid model employing two nurses on the efficient rescue operation time and the efficient resuscitation time for major burn patients. A two-nurse model of first aid was designed for major burn patients. The model includes a division of labor between the first aid nurses and the re-organization of emergency carts. The clinical effectiveness of the process was examined in a retrospective chart review of 156 cases of major burn patients, experiencing shock and low blood volume, who were admitted to the intensive care unit of the department of burn surgery between November 2009 and June 2013. Of the 156 major burn cases, 87 patients who received first aid using the double personnel model were assigned to the test group and the 69 patients who received first aid using the standard first aid model were assigned to the control group. The efficient rescue operation time and the efficient resuscitation time for the patients were compared between the two groups. Student's t tests were used to the compare the mean difference between the groups. Statistically significant differences between the two groups were found on both measures (P's first aid model based on scientifically validated procedures and a reasonable division of labor can shorten the efficient rescue operation time and the efficient resuscitation time for major burn patients. Given these findings, the model appears to be worthy of clinical application.

[Epidemiological study on thalassemia among the children of 0 - 7 years old among the six ethnic groups in Xishuangbanna and Dehong of Yunnan province].

Science.gov (United States)

Zhao, Zhong-Ming; Yao, Li-Qin; Fan, Li-Mei; Zou, Tuan-Biao; Chen, Qian; Hu, Li-Sha; Yang, Fa-Bin; Liu, Jin-Tao; Wang, Xing-Tian

2011-04-01

To investigate the prevalence rate of thalassemia among children of 0 - 7 years old, from six ethnic groups in Xishuangbanna and Dehong, Yunnan province. 4973 blood samples from children under 7 years old were automatically undergone blood cell count, red cell osmotic fragility and hemoglobin electrophoresis testings. The incidence rates of thalassaemia, β-thalassemia was 37.4%, and α-thalassaemia were 22.6% and 14.7% respectively. The thalassaemia incidence rates were significantly different among age groups but not in gender. The incidence of α-thalassaemia was decreasing along with the increase of age, while the incidence of β-thalassaemia was increasing along with the increase of age. Xishuangbanna had the higher incidence than in Dehong and the differences were significant between counties, The incidence of thalassemia of Mengla ranked the first (52.2%) in Xishuangbanna, The differences between different regions and different nationalities were significant, with β-thalassemia of Achang ranked the first (40.6%), The incidence of α-thalassemia among Han ranked the first as 45.5% while α-thalassaemia and β-thalassemia were different in regions. α-thalassaemia and β-thalassemia were significantly different between different ethnic people in the same regions. Multiple factor analysis showed that region seemed to be a risk factor and the mother's ethnicity was a protective factor and dependent variable on thalassaemia. The incidence of thalassaemia in Yunnan Xishuangbanna and Dehong was high among children under the age of 7 and were related to ethnic and regional differences in the areas. Specific genes were proliferated along with the extension of time. Our data provided valuable information on prevention and genetic studies on thalassaemia in the minorities of Xishuangbanna and Dehong in Yunnan province.

Non-major bleeding with apixaban versus warfarin in patients with atrial fibrillation

NARCIS (Netherlands)

Bahit, M.C.; Lopes, R.D.; Wojdyla, D.M.; Held, C.; Hanna, M.; Vinereanu, D.; Hylek, E.M.; Verheugt, F.W.; Goto, S.; Alexander, J.H.; Wallentin, L.; Granger, C.B.

2017-01-01

OBJECTIVE: We describe the incidence, location and management of non-major bleeding, and assess the association between non-major bleeding and clinical outcomes in patients with atrial fibrillation (AF) receiving anticoagulation therapy enrolled in Apixaban for Reduction in Stroke and other

Comparison of right and left side heart functions in patients with thalassemia major, patients with thalassemia intermedia, and control group.

Science.gov (United States)

Noori, Noormohammad; Mohamadi, Mehdi; Keshavarz, Kambiz; Alavi, Seyed Mostafa; Mahjoubifard, Maziar; Mirmesdagh, Yalda

2013-01-01

Heart disease is the main cause of mortality and morbidity in patients with beta thalassemia, rendering its early diagnosis vital. We studied and compared echocardiographic findings in patients with beta thalassemia major, patients with beta thalassemia intermedia, and a control group. Eighty asymptomatic patients with thalassemia major and 22 asymptomatic cases with thalassemia intermedia (8-25 years old) were selected from those referred to Ali Asghar Hospital (Zahedan-Iran) between June 2008 and June 2009. Additionally, 80 healthy individuals within the same age and sex groups were used as controls. All the individuals underwent echocardiography, the data of which were analyzed with the Student t-test. The mean value of the pre-ejection period/ejection time ratio of the left ventricle during systole, the diameter of the posterior wall of the left ventricle during diastole, the left and right isovolumic relaxation times, and the right myocardial performance index in the patients with beta thalassemia major and intermedia increased significantly compared to those of the controls, but the other parameters were similar between the two patient groups. The mean values of the left and right pre-ejection periods, left ventricular end systolic dimension, and left isovolumic contraction time in the patients with thalassemia intermedia increased significantly compared to those of the controls. In the left side, myocardial performance index, left ventricular mass index, isovolumic contraction time, and deceleration time exhibited significant changes between the patients with thalassemia major and those with thalassemia intermedia, whereas all the echocardiographic parameters of the right side were similar between these two groups. The results showed that the systolic and diastolic functions of the right and left sides of the heart would be impaired in patients with thalassemia major and thalassemia intermedia. Consequently, serial echocardiography is suggested in

Immunological Evaluation of -Thalassemia Major Patients Receiving Oral Iron Chelator Deferasirox

International Nuclear Information System (INIS)

Aleem, A.; Alsaleh, K.; Algahtani, F.; Momen, A. A.; Shakoor, Z.; Iqbal, Z.

2014-01-01

Objective: To determine the immune abnormalities and occurrence of infections in transfusion-dependent -thalassemia major patients receiving oral iron chelator deferasirox (DFX). Study Design: An observational study. Place and Duration of Study: Hematology Clinics, King Khalid University Hospital, Riyadh, Saudi Arabia, from July to December 2010. Methodology: Seventeen patients with -thalassemia major (12 females, median age 26 years) receiving deferasirox (DFX) for a median duration of 27 months were observed for any infections and had their immune status determined. Immune parameters studied included serum immunoglobulins and IgG subclasses, serum complement (C3 and C4) and anti-nuclear antibody (ANA) level, total B and T-lymphocytes, CD4+ and CD8+ counts, CD4+/CD8+ ratio, and natural killer (NK) cells. Immunological parameters of the patients were compared with age, gender, serum ferritin level and splenectomy status. Lymphocyte subsets were also compared with age and gender matched normal controls. Results: A considerable reduction in serum ferritin was achieved by DFX from a median level of 2528 to 1875 mol/l. Serum IgG levels were increased in 7 patients. Low C4 levels were found in 9 patients. Total B and T-lymphocytes were increased in 14 patients each, while CD4+, CD8+ and NK cells were increased in 13, 12 and 11 patients respectively. Absolute counts for all lymphocyte subsets were significantly higher compared to the normal controls (p=0.05 for all parameters). Raised levels of IgG were associated with older age, female gender, splenectomized status and higher serum ferritin levels but this did not reach statistical significance except for the higher ferritin levels (p=0.044). Increased tendency to infections was not observed. Conclusion: Patients with -thalassemia major receiving DFX exhibited significant immune abnormalities. Changes observed have been described previously, but could be related to DFX. The immune abnormalities were not associated with

Resilience Modulators and Overburden in Major Caregivers of Advanced Cancer Patients

Directory of Open Access Journals (Sweden)

Anay González Guerra

2017-03-01

Full Text Available Foundation: the caregiver plays an important role in helping and supporting a patient with cancer, but at the same time has the emotional and work burden which this work presupposes. Objective: to determine the resilience modulating factors and the degree of burden in major caregivers of advanced cancer patients. Method: a descriptive study was developed at the Area III Policlinic Cienfuegos during the period from December 2012 to March 2013. The universe was constituted by 25 primary caregivers of patients in an advanced stage of the disease. The studied variables were: sex, age, scholarship, occupation, marital status, kinship, time to patient care, self-esteem, optimism, emotional intelligence, and burden. The techniques used were: questionnaire and test of emotional intelligence, Coopersmith self-esteem inventory, caregiver burden scale by Zarit. Results: ages between 51 and 72 years predominated 48 %, 88 % were female, 40 % had elementary school level and 48 % were married, 72 % were housewives, 44 % were patients spouses, 52 % less than a year of care giving. 60 % had an intense burden, 48 % low level of self-esteem and an intense burden. 58.3 % of caregivers with low emotional intelligence had an intense burden. Conclusion: resilience modulators, self-esteem and emotional intelligence determine the level of burden suffered by major caregivers of advanced cancer patients.

Complex preimplantation genetic diagnosis for beta-thalassaemia, sideroblastic anaemia, and human leukocyte antigen (HLA)-typing.

Science.gov (United States)

Kakourou, Georgia; Vrettou, Christina; Kattamis, Antonis; Destouni, Aspasia; Poulou, Myrto; Moutafi, Maria; Kokkali, Georgia; Pantos, Konstantinos; Davies, Stephen; Kitsiou-Tzeli, Sophia; Kanavakis, Emmanuel; Traeger-Synodinos, Joanne

2016-01-01

Preimplantation genetic diagnosis (PGD) to select histocompatible siblings to facilitate curative haematopoeitic stem-cell transplantation (HSCT) is now an acceptable option in the absence of an available human leukocyte antigen (HLA) compatible donor. We describe a case where the couple who requested HLA-PGD, were both carriers of two serious haematological diseases, beta-thalassaemia and sideroblastic anaemia. Their daughter, affected with sideroblastic anaemia, was programmed to have HSCT. A multiplex-fluorescent-touchdown-PCR protocol was optimized for the simultaneous amplification of: the two HBB-gene mutated regions (c.118C> T, c.25-26delAA), four short tandem repeats (STRs) in chr11p15.5 linked to the HBB gene, the SLC25A38 gene mutation (c.726C > T), two STRs in chr3p22.1 linked to the SLC25A38 gene, plus eleven informative STRs for HLA-haplotyping (chr6p22.1-21.3). This was followed by real-time nested PCR and high-resolution melting analysis (HRMA) for the detection of HBB and SLC25A38 gene mutations, as well as the analysis of all STRs on an automatic genetic analyzer (sequencer). The couple completed four clinical in vitro fertilization (IVF)/PGD cycles. At least one matched unaffected embryo was identified and transferred in each cycle. A twin pregnancy was established in the fourth PGD cycle and genotyping results at all loci were confirmed by prenatal diagnosis. Two healthy baby girls were delivered at week 38 of pregnancy. The need to exclude two familial disorders for HLA-PGD is rarely encountered. The methodological approach described here is fast, accurate, clinically-validated, and of relatively low cost.

Regional cerebral glucose metabolism in systemic lupus erythematosus patients with major depressive disorder.

Science.gov (United States)

Saito, Tomoyuki; Tamura, Maasa; Chiba, Yuhei; Katsuse, Omi; Suda, Akira; Kamada, Ayuko; Ikura, Takahiro; Abe, Kie; Ogawa, Matsuyoshi; Minegishi, Kaoru; Yoshimi, Ryusuke; Kirino, Yohei; Ihata, Atsushi; Hirayasu, Yoshio

2017-08-15

Depression is frequently observed in patients with systemic lupus erythematosus (SLE). Neuropsychiatric SLE (NPSLE) patients often exhibit cerebral hypometabolism, but the association between cerebral metabolism and depression remains unclear. To elucidate the features of cerebral metabolism in SLE patients with depression, we performed brain 18F-fluoro-d-glucose positron emission tomography (FDG-PET) on SLE patients with and without major depressive disorder. We performed brain FDG-PET on 20 SLE subjects (5 male, 15 female). The subjects were divided into two groups: subjects with major depressive disorder (DSLE) and subjects without major depressive disorder (non-DSLE). Cerebral glucose metabolism was analyzed using the three-dimensional stereotactic surface projection (3D-SSP) program. Regional metabolism was evaluated by stereotactic extraction estimation (SEE), in which the whole brain was divided into segments. Every SLE subject exhibited cerebral hypometabolism, in contrast to the normal healthy subjects. Regional analysis revealed a significantly lower ER in the left medial frontal gyrus (p=0.0055) and the right medial frontal gyrus (p=0.0022) in the DSLE group than in the non-DSLE group. Hypometabolism in the medial frontal gyrus may be related to major depressive disorder in SLE. Larger studies are needed to clarify this relationship. Copyright © 2017 Elsevier B.V. All rights reserved.

A Comparison of Sexual Dysfunctions in Female Patients with Major Depressive Disorder and Panic Disorder

OpenAIRE

Tonguç Demir Berkol; Süheyla Doðan Bulut; Esra Alataþ; Dicle Görkem; Esra Çavdar; Ýlker Özyýldýrým

2014-01-01

Objective: The aim of this study is assessment of sexual dysfunction in female patients with major depressive disorder and panic disorder and compare the two groups. Methods: Total 76 female patients with primary diagnosis of major depressive disorder ( 46 patients) and panic disorder ( 30 patients) according to DSM-IV, who is sexually active and not use psychotropic medication were inclued. Sociodemographic data aqcusition form and the Arizona Sexual Experiences Scale (ASEX) were adminis...

Epidemiology and clinical impact of major comorbidities in patients with COPD

Directory of Open Access Journals (Sweden)

Smith MC

2014-08-01

Full Text Available Miranda Caroline Smith,1 Jeremy P Wrobel2 1Respiratory Medicine, Royal Perth Hospital, Perth, WA, Australia; 2Advanced Lung Disease Unit, Royal Perth Hospital, Perth, WA, Australia Abstract: Comorbidities are frequent in chronic obstructive pulmonary disease (COPD and significantly impact on patients' quality of life, exacerbation frequency, and survival. There is increasing evidence that certain diseases occur in greater frequency amongst patients with COPD than in the general population, and that these comorbidities significantly impact on patient outcomes. Although the mechanisms are yet to be defined, many comorbidities likely result from the chronic inflammatory state that is present in COPD. Common problems in the clinical management of COPD include recognizing new comorbidities, determining the impact of comorbidities on patient symptoms, the concurrent treatment of COPD and comorbidities, and accurate prognostication. The majority of comorbidities in COPD should be treated according to usual practice, and specific COPD management is infrequently altered by the presence of comorbidities. Unfortunately, comorbidities are often under-recognized and under-treated. This review focuses on the epidemiology of ten major comorbidities in patients with COPD. Further, we emphasize the clinical impact upon prognosis and management considerations. This review will highlight the importance of comorbidity identification and management in the practice of caring for patients with COPD. Keywords: cardiovascular disease, prevalence, mortality, chronic bronchitis, emphysema

Major stroke in a 19-year-old patient with a univentricular heart

DEFF Research Database (Denmark)

Riemann, Mads; Idorn, Lars; Wagner, Aase

2013-01-01

Patients with univentricular heart malformations are at increased risk of suffering from thromboembolic events. We present a case of a 19-year-old woman born with a univentricular heart who suffered a major stroke while being treated with only salicylic acid. At least 20% of patients...

Splenectomized versus non-splenectomized patients with thalassemia major: Echocardiographic comparison

International Nuclear Information System (INIS)

Morsy, Mohamed-Mofeed F.; Ahmed, Ali A.; Al-Najjar, Abdulhameed A.; Almuzainy, Ibrahim S.; Alhawsawi, Zakria M.; Alserafi, Mona H.

2008-01-01

Objective was to study the effect of splenectomy in patients with thalassemia major on the cardiovascular system through echocardiographic study. A prospective, cross-sectional study was carried out from December 2006 to December 2007. Patients from the Thalassemia Center in the Maternity and Children's Hospital, Madina, Kingdom of Saudi Arabia, were screened by means of history, physical examination, laboratory studies and echocardiography. Fifty-seven patients were studied: 36 were non-splenectomized, while 21 were surgically splenectomized. The 2 study groups were well matched for age, gender, height and weight. The total amount of blood given previous year (6577+-206.9 ml versus 5390.5+-220.2 ml, p=0.0005) and the annual transfusion index (200.9+-11.3 ml versus 134.1+-7.3, p=0.0001) were significantly lower in the splenectomized group. There was no significant difference between 2 groups regarding laboratory studies. Left ventricular systolic function shows no difference regarding fraction shortening between the 2 groups. The mitral valve E/A ratio was significantly higher in the splenectomized group (1.6+-0.2 versus1.4+-0.2, p=0.02). The pulmonary artery pressure was higher in the splenectomized group (34.2+-9.1 versus 20.8+-9.2 mm Hg, p=0.0001). There was a significantly higher number of patients with pulmonary hypertension in the splenectomized group (14[66.7%] versus 6[1.7%], p=0.0004). Splenectomized patients with thalassemia major are at high risk of having impaired diastolic left ventricular function and pulmonary hypertension. (author)

Volumetric MRI analysis of the amygdala and the hippocampus in patients with major depression

International Nuclear Information System (INIS)

Xia Jun; Zhou Yicheng; Zhang Jingfeng; Yang Bo; Xia Liming; Wang Chengyuan; Chen Jun

2005-01-01

Objective: To study the MRI volume of the amygdala and hippocampus in patients with major depression. Methods: Quantitative MRI of the amygdala and hippocampus was studied in 22 patients with major depression and compared with 13 age-matched controls. Results: Both groups exhibited similar significant hippocampal asymmetry (left smaller than right). The volume of the bilateral hippocampus was significantly smaller in the patient group than that in the controls (left: t=9.96, P<0.01; right: t=11.88, P<0.01). The right amygdala was smaller in the patient group than that in the control group (t=5.50, P<0.01), No correlation was found between the hippocampal volume abnormalities and the course of disease. Conclusion: These findings support the hypothesis that the hippocampus and amygdala within limbic-cortical networks may play a crucial role in the pathogenesis of major depression. (authors)

Patients With Diabetic Foot Disease Fear Major Lower-Extremity Amputation More Than Death.

Science.gov (United States)

Wukich, Dane K; Raspovic, Katherine M; Suder, Natalie C

2018-02-01

The aim of this study was to identify the most-feared complications of diabetes mellitus (DM), comparing those with diabetic foot pathology with those without diabetic foot pathology. We determined the frequency of patients ranking major lower-extremity amputation (LEA) as their greatest fear in comparison to blindness, death, diabetic foot infection (DFI), or end-stage renal disease (ESRD) requiring dialysis. We further categorized the study group patients (N = 207) by their pathology such as diabetic foot ulcer (DFU), Charcot neuroarthropathy, foot infection, or acute neuropathic fractures and dislocations. The control group (N = 254) was comprised of patients with diabetes who presented with common non-diabetes-related foot pathology. A total of 461 patients were enrolled in this study and included 254 patients without diabetic foot complications and 207 patients with diabetic foot problems. When comparing patients with and without diabetic disease, no significant differences were observed with regard to their fear of blindness, DFI, or ESRD requiring dialysis. Patients with diabetic foot disease (61 of 207, 31.9%) were 136% more likely (odds ratio [OR] = 2.36; 95% CI = 1.51-3.70; P = .002] to rank major LEA as their greatest fear when compared with diabetic patients without foot disease (42 of 254, 16.5%) and were 49% less likely (OR = 0.51; 95% CI = 0.34-0.79; P = .002) to rank death as their greatest fear compared with patients without diabetic foot disease. Patients with diabetic foot pathology fear major LEA more than death, foot infection, or ESRD. Variables that were associated with ranking LEA as the greatest fear were the presence of a diabetic-related foot complication, duration of DM ≥10 years, insulin use, and the presence of peripheral neuropathy. Level II: Prospective, Case controlled study.

Haematopoietic tissue presenting as a sphenoid sinus mass: case report

International Nuclear Information System (INIS)

Joseph, M.; Rajshekhar, V.; Chandy, M.J.

2000-01-01

We report an incidentally discovered mass in the sphenoid sinus in a patient with beta thalassaemia and sickle-cell disease which proved to be an isolated site of extramedullary haematopoiesis in the skull. (orig.)

Haematopoietic tissue presenting as a sphenoid sinus mass: case report

Energy Technology Data Exchange (ETDEWEB)

Joseph, M.; Rajshekhar, V.; Chandy, M.J. [Dept. of Neurological Sciences, Christian Medical College Hospital, Vellore (India)

2000-02-01

We report an incidentally discovered mass in the sphenoid sinus in a patient with beta thalassaemia and sickle-cell disease which proved to be an isolated site of extramedullary haematopoiesis in the skull. (orig.)

Correlates of late-life major depression: a comparison of urban and rural primary care patients.

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Friedman, Bruce; Conwell, Yeates; Delavan, Rachel L

2007-01-01

The objective of this study was to determine whether factors associated with depression differ between elderly residents of rural and urban areas. The research design was cross-sectional and observational. The study subjects consisted of 926 Medicare primary care patients (650 urban and 276 rural) who were age 65+ and cognitively intact and had enrolled in a randomized, controlled Medicare demonstration. Major depression was identified by the Mini International Neuropsychiatric Interview. A logistic regression model was estimated that included a rural-urban indicator variable, additional independent variables, and interaction terms between the rural-urban indicator and independent variables that were significant at p Reporting 0-1 close friends (odds ratio [OR]: 6.86; 95% confidence interval [CI]: 2.18-21.58), 2+ emergency room visits during the past 6 months (OR: 4.00; 95% CI: 1.19-13.43), and more financial strain (OR: 1.50; 95% CI: 1.01-2.23) were associated with significantly higher likelihood of major depression among rural as compared with urban patients. The SF-36 Physical Component Summary score had a curvilinear relationship with major depression and was higher for urban patients. The predicted probability for major depression is lower for the rural patients when financial strain is low, about the same for rural and urban patients when strain is intermediate, and higher for rural patients when strain is high. Clinicians in rural areas should be vigilant for major depression among patients with very few close friends, several recent emergency department visits, and financial strain.

Thalassaemic osteoarthropathy treated with radiotherapy

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Davies, A.N.

1993-01-01

Patients with beta thalassaemia may develop a specific osteoarthropathy involving the feet. A number of different treatments for this condition have been tried, including rest, analgesia and hypertransfusion. We report a case of a patient with thalassaemic osteoarthropathy who responded to radiotherapy after failing conventional treatment. (author)

The association of major depressive episode and personality traits in patients with fibromyalgia

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Danyella de Melo Santos

2011-01-01

Full Text Available INTRODUCTION: Personality traits have been associated with primary depression. However, it is not known whether this association takes place in the case of depression comorbid with fibromyalgia. OBJECTIVE: The authors investigated the association between a current major depressive episode and temperament traits (e.g., harm avoidance. METHOD: A sample of 69 adult female patients with fibromyalgia was assessed with the Temperament and Character Inventory. Psychiatric diagnoses were assessed with the Mini-International Neuropsychiatric Interview severity of depressive symptomatology with the Beck Depression Inventory, and anxiety symptomatology with the IDATE-state and pain intensity with a visual analog scale. RESULTS: A current major depressive episode was diagnosed in 28 (40.5% of the patients. They presented higher levels of harm avoidance and lower levels of cooperativeness and self-directedness compared with non-depressed patients, which is consistent with the Temperament and Character Inventory profile of subjects with primary depression. However, in contrast to previous results in primary depression, no association between a major depressive episode and self-transcendence was found. CONCLUSIONS: The results highlight specific features of depression in fibromyalgia subjects and may prove important for enhancing the diagnosis and prognosis of depression in fibromyalgia patients.

Clinical outcomes of patients with major bleeding after primary coronary intervention for acute ST-segment elevation myocardial infarction

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Zheng Hongchao; Zhang Qi; Zhang Ruiyan; Hu Jian; Yang Zhenkun; Zhang Jiansheng; Shen Weifeng

2009-01-01

Objective: To evaluate the clinical outcomes of patients complicated with major bleeding after primary coronary intervention (PCI) for acute ST-segment elevation myocardial infarction (STEMI). Methods: During the period of January 2004-January 2008, primary PCI was performed in 412 consecutive patients with acute STEMI at Shanghai Ruijin Hospital. The clinical data were retrospectively analyzed. Major adverse cardiac events (MACE), including death, reoccurrence of myocardial infarction and target vessel revascularization, in patients with major bleeding were compared with that in patients without major bleeding. Results: Compared to patients without bleeding, the patients with bleeding were older (70.0 ± 8.9 years vs 64.9 ± 12.7 years, P=0.04), mainly the females (51.9% vs 23.1%, P=0.001) and treated more often with glycoprotein (GP) IIb / IIIa receptor inhibitor (88.9% vs 69.4%, P=0.03) or intra-aortic balloon pump (7.4% vs 1.3%, P=0.02). In-hospital and one-year MACE rate in the patients with bleeding was 18.5% and 37.0% respectively,which were significantly higher than that in the patients without bleeding (5.7% and 14.3%, with P=0.008 and P=0.002, respectively). Multivariate analysis indicated that patient aged over 70 years, feminine gender and use of GP IIb/IIIa receptor inhibitor were independent predictors for the occurrence of major bleeding. The occurrence of major bleeding after primary PCI was significantly correlated with MACE occurred within one year after the procedure (OR 2.79, 95% CI: 2.21-5.90, P<0.001). Conclusion: In patients with acute STEMI, the occurrence of major bleeding after primary PCI is closely linked to the increased MACE rate within one year after the treatment.Feminine gender, aged patient and use of GPIIb/IIIa receptor inhibitor are independent predictors to increase the danger of major bleeding. (authors)

GROWTH AND ENDOCRINE FUNCTION IN TUNISIAN THALASSEMIA MAJOR PATIENTS

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Naouel GUIRAT

2018-05-01

Full Text Available Beta-thalassemia major (TM is among prevalent hereditary disorders imposing high expenses on health-care system worldwide. The patient’s survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity on various organs including endocrine glands. This article provides an overview of  endocrine disorders in beta-TM patients. This single center investigation enrolled 28 beta-TM patients (16 males, 12 females  regularly transfused with packed red cell since early years of life. For each patient were determined: age, sex, number of transfusions received, history of splenectomy and anthropometric parameters. Evaluation for hormonal status including growth, gonadal, thyroid, adrenal cortex, and parathyroid glands was done for all patients. Dual energy X-ray absorptiometry was used to diagnose osteoporosis. Assessment of iron overload status was performed by measuring the serum ferritin concentration and the results of magnetic resonance imaging T2*. Growth retardation was found in 16 of the 28 studied patients (57 %.Thirteen among them had delayed puberty. Spontaneous puberty was achieved in 16 cases. Growth hormone (GH deficiency was found in 10 cases (35 %. Seventeen among the studied patients (60 % developed disorders of glucose homeostasis. Subclinical hypothyroidism was found in six patients (21 %. Intensive chelation therapy had allowed the reversibility of this complication in five cases. Adrenal Insufficiency was found in 9 cases (32%. Hypoparathyroidism has occurred in one case. Ten of the 28 studied patients had osteoporosis (35%. Twenty-three of the 28 studied patients (82% had at least one endocrine complication.

Salivary alpha-amylase and cortisol responsiveness following electrical stimulation stress in major depressive disorder patients.

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Tanaka, Yoshihiro; Ishitobi, Yoshinobu; Maruyama, Yoshihiro; Kawano, Aimi; Ando, Tomoko; Okamoto, Shizuko; Kanehisa, Masayuki; Higuma, Haruka; Ninomiya, Taiga; Tsuru, Jusen; Hanada, Hiroaki; Kodama, Kensuke; Isogawa, Koichi; Akiyoshi, Jotaro

2012-03-30

Major depressive disorder (MDD) is often associated with dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis by chronic stress. In comparison, psychosocial stress-induced activation of salivary α-amylase (sAA) functions as a marker of sympathoadrenal medullary system (SAM) activity. However, in contrast to salivary cortisol, sAA has been less extensively studied in MDD patients. The present study measured sAA and salivary cortisol levels in patients with MDD. The authors determined Profile of Mood State (POMS) and State-Trait anxiety Inventory (STAI) scores, Heart Rate Variability (HRV), and sAA and salivary cortisol levels in 88 patients with MDD and 41 healthy volunteers following the application of electrical stimulation stress. Patients with major depressive disorder were 8 points or more on Hamilton Depression Scale (HAM-D) scores. Tension-Anxiety, Depression-Dejection, Anger-Hostility, Fatigue, and Confusion scores in patients with major depressive disorder were significantly increased compared to healthy controls. In contrast, Vigor scores in patients with MDD were significantly decreased compared with healthy controls. There was no difference in heart rate variability measures between MDD patients and healthy controls. The threshold of electrical stimulation applied in MDD patients was lower than that in healthy controls. SAA levels in female MDD patients were significantly elevated relative to controls both before and after electrical stimulation. Finally, there were no differences in salivary cortisol levels between major depressive patients and controls. In the present study only three time points were explored. Furthermore, the increased secretion of sAA before and after stimulation could allude to an increased responsiveness of novel and uncontrollable situations in patients with MDD. These preliminary results suggest that sAA might be a useful biological marker of MDD. Copyright © 2011 Elsevier Inc. All rights reserved.

Effects of blood transfusion on exercise capacity in thalassemia major patients.

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Daniela Benedetto

Full Text Available Anemia has an important role in exercise performance. However, the direct link between rapid changes of hemoglobin and exercise performance is still unknown.To find out more on this topic, we studied 18 beta-thalassemia major patients free of relevant cardiac dysfunction (age 33.5±7.2 years,males = 10. Patients performed a maximal cardiopulmolmonary exercise test (cycloergometer, personalized ramp protocol, breath-by-breath measurements of expired gases before and the day after blood transfusion (500 cc of red cell concentrates. After blood transfusion, hemoglobin increased from 10.5±0.8 g/dL to 12.1±1.2 (p<0.001, peak VO2 from 1408 to 1546mL/min (p<0.05, and VO2 at anaerobic threshold from 965 to 1024mL/min (p<0.05. No major changes were observed as regards heart and respiratory rates either at peak exercise or at anaerobic threshold. Similarly, no relevant changes were observed in ventilation efficiency, as evaluated by the ventilation vs. carbon dioxide production relationship, or in O2 delivery to the periphery as analyzed by the VO2 vs. workload relationship. The relationship between hemoglobin and VO2 changes showed, for each g/dL of hemoglobin increase, a VO2 increase = 82.5 mL/min and 35 mL/min, at peak exercise and at anaerobic threshold, respectively. In beta-thalassemia major patients, an acute albeit partial anemia correction by blood transfusion determinates a relevant increase of exercise performance, observed both at peak exercise and at anaerobic threshold.

Prolidase activity and oxidative stress in patients with major depressive disorder.

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Kokacya, Mehmet Hanifi; Bahceci, Bulent; Bahceci, Ilkay; Dilek, Aziz Ramazan; Dokuyucu, Recep

2014-12-01

The aim of the current study was to determine whether the serum prolidase levels are associated with the etiopathogenesis of depression. This study included 29 patients with major depressive disorder (MDD), who were consecutively recruited from the psychiatric outpatient clinic, and 30 healthy individuals recruited from the general community. Each patient underwent a detailed diagnostic evaluation by two psychiatrists using the Structured Clinical Interview for DSM-IV (SCID-I). Serum prolidase activity and oxidative parameters were measured in the patient and control groups. The severity of depressive symptoms was assessed using the Hamilton Depression Rating Scale. Serum prolidase level was significantly higher in patients with MDD compared to healthy subjects (pStress Index (OSI) were also significantly higher in patients with MDD (pstress in patients with MDD. Increased serum prolidase levels in patients with MDD may be interpreted as the interaction of prolidase activity, glutamate transmission and oxidative stress. It is suggested that prolidase activity is involved in the etiopathogenesis of depressive disorder.

Personality styles in patients with fibromyalgia, major depression and healthy controls

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Stiles Tore C

2007-03-01

Full Text Available Abstract Background The fibromyalgia syndrome (FMS is suggested to be a manifestation of depression or affective spectrum disorder. We measured the cognitive style of patients with FMS to assess personality styles in 44 patients with fibromyalgia syndrome (FMS by comparing them with 43 patients with major depressive disorder (MDD and 41 healthy controls (HC. Methods Personality styles were measured by the Sociotropy and Autonomy Scale (SAS and the Dysfunctional Attitude Scale (DAS. The Structured Clinical interview for DSM Axis I was applied to Axis I disorders, while the Beck Depression Inventory was used to measure depression severity. Results Patients with FMS in general have a sociotropic personality style similar to patients with MDD, and different from HC, but FMS patients without a lifetime history of MDD had a cognitive personality style different from patients with MDD and similar to HC. Conclusion These findings suggest that a depressotypic personality style is related to depressive disorder, but not to FMS.

Bacterial genus is a risk factor for major amputation in patients with diabetic foot.

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Cardoso, Natália Anício; Cisneiros, Lígia DE Loiola; Machado, Carla Jorge; Cenedezi, Juliana Merlin; Procópio, Ricardo Jayme; Navarro, Túlio Pinho

2017-01-01

to evaluate whether bacterial genus is a risk factor for major amputation in patients with diabetic foot and infected ulcer. we conducted a case-control, observational study of 189 patients with infected ulcers in diabetic feet admitted to the Vascular Surgery Service of the Risoleta Tolentino Neves Hospital, from January 2007 to December 2012. The bacteriological evaluation was performed in deep tissue cultures from the lesions and amputation was considered major when performed above the foot'smiddle tarsus. the patients'mean age was 61.9±12.7 years; 122 (64.6%) were men. The cultures were positive in 86.8%, being monomicrobial in 72% of the cases. In patients with major amputation, Acinetobacter spp. (24.4%), Morganella spp. (24.4%), Proteus spp. (23.1%) and Enterococcus spp. (19.2%) were the most frequent types of bacteria. The most commonly isolated species were Acinetobacter baumannii, Morganella morganii, Pseudomonas aeruginosa and Proteus mirabilis. As predictors of major amputation, we identified the isolation of the generaAcinetobacter spp. and Klebsiella spp., serum creatinine ≥1.3mg/dl and hemoglobin creatinina ≥1,3mg/dl e de hemoglobina <11g/dl. os gêneros bacterianos Acinetobacter spp. e Klebsiella spp. identificados nas úlceras infectadas dos pacientes com pé diabético associaram-se a maior incidência de amputação maior.

12th International Conference on Thalassemia and the Haemoglobinopathies, 11-14 May 2011, Antalya, Turkey

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Guest Editors: Aurelio Maggio, Duran Canatan, Androulla Eleftheriou

2011-05-01

Full Text Available This abstract book contains all abstracts presented to the 12th International Conference on Thalassaemia and Other Haemoglobinopathies, and the 14th TIF Conference for Patients and Parents, 11-14 May 2011, Antalya, Turkey.

Multifilament Cable Wire versus Conventional Wire for Sternal Closure in Patients Undergoing Major Cardiac Surgery

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You Na Oh

2015-08-01

Full Text Available Background: Stainless steel wiring remains the most popular technique for primary sternal closure. Recently, a multifilament cable wiring system (Pioneer Surgical Technology Inc., Marquette, MI, USA was introduced for sternal closure and has gained wide acceptance due to its superior resistance to tension. We aimed to compare conventional steel wiring to multifilament cable fixation for sternal closure in patients undergoing major cardiac surgery. Methods: Data were collected retrospectively on 1,354 patients who underwent sternal closure after major cardiac surgery, using either the multifilament cable wiring system or conventional steel wires between January 2009 and October 2010. The surgical outcomes of these two groups of patients were compared using propensity score matching based on 18 baseline patient characteristics. Results: Propensity score matching yielded 392 pairs of patients in the two groups whose baseline profiles showed no significant differences. No significant differences between the two groups were observed in the rates of early mortality (2.0% vs. 1.3%, p=0.578, major wound complications requiring reconstruction (1.3% vs. 1.3%, p＞0.99, minor wound complications (3.6% vs. 2.0%, p=0.279, or mediastinitis (0.8% vs. 1.0%, p=1.00. Patients in the multifilament cable group had fewer sternal bleeding events than those in the conventional wire group, but this tendency was not statistically significant (4.3% vs. 7.4%, p=0.068. Conclusion: The surgical outcomes of sternal closure using multifilament cable wires were comparable to those observed when conventional steel wires were used. Therefore, the multifilament cable wiring system may be considered a viable option for sternal closure in patients undergoing major cardiac surgery.

Multifilament Cable Wire versus Conventional Wire for Sternal Closure in Patients Undergoing Major Cardiac Surgery.

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Oh, You Na; Ha, Keong Jun; Kim, Joon Bum; Jung, Sung-Ho; Choo, Suk Jung; Chung, Cheol Hyun; Lee, Jae Won

2015-08-01

Stainless steel wiring remains the most popular technique for primary sternal closure. Recently, a multifilament cable wiring system (Pioneer Surgical Technology Inc., Marquette, MI, USA) was introduced for sternal closure and has gained wide acceptance due to its superior resistance to tension. We aimed to compare conventional steel wiring to multifilament cable fixation for sternal closure in patients undergoing major cardiac surgery. Data were collected retrospectively on 1,354 patients who underwent sternal closure after major cardiac surgery, using either the multifilament cable wiring system or conventional steel wires between January 2009 and October 2010. The surgical outcomes of these two groups of patients were compared using propensity score matching based on 18 baseline patient characteristics. Propensity score matching yielded 392 pairs of patients in the two groups whose baseline profiles showed no significant differences. No significant differences between the two groups were observed in the rates of early mortality (2.0% vs. 1.3%, p=0.578), major wound complications requiring reconstruction (1.3% vs. 1.3%, p>0.99), minor wound complications (3.6% vs. 2.0%, p=0.279), or mediastinitis (0.8% vs. 1.0%, p=1.00). Patients in the multifilament cable group had fewer sternal bleeding events than those in the conventional wire group, but this tendency was not statistically significant (4.3% vs. 7.4%, p=0.068). The surgical outcomes of sternal closure using multifilament cable wires were comparable to those observed when conventional steel wires were used. Therefore, the multifilament cable wiring system may be considered a viable option for sternal closure in patients undergoing major cardiac surgery.

Major surgery in an osteosarcoma patient refusing blood transfusion: case report.

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Dhanoa, Amreeta; Singh, Vivek A; Shanmugam, Rukmanikanthan; Rajendram, Raja

2010-11-08

We describe an unusual case of osteosarcoma in a Jehovah's Witness patient who underwent chemotherapy and major surgery without the need for blood transfusion. This 16-year-old girl presented with osteosarcoma of the right proximal tibia requiring proximal tibia resection, followed by endoprosthesis replacement. She was successfully treated with neoadjuvant chemotherapy and surgery with the support of haematinics, granulocyte colony-stimulating factor, recombinant erythropoietin and intraoperative normovolaemic haemodilution. This case illustrates the importance of maintaining effective, open communication and exploring acceptable therapeutic alternative in the management of these patients, whilst still respecting their beliefs.

Major depressive disorder: a qualitative study on the experiences of Iranian patients.

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Amini, Kourosh; Negarandeh, Reza; Cheraghi, Mohammad Ali; Eftekhar, Mehrdad

2013-09-01

Major depressive disorder (MDD) is one the most common mental disorders; it affects about 5-10% of the world population. This study explores the experiences of people with major depressive disorder in Zanjan, Iran. In order to identify recurring themes and patterns in individuals' experiences of major depressive disorder, semi-structured interviews with 18 patients were recorded and transcribed verbatim. The transcripts were then analyzed based on conventional qualitative content analysis. Five main categories emerged. The first category was called emotional paralysis and included the subcategories feeling severely depressed; feeling anxious; feeling impatient and irritable; and having dyshedonia. The second category was disturbance of thinking and was comprised of the subcategories of preoccupation, instable spiritual beliefs, and guilt. Cognitive decline was the third identified category and was further divided into subcategories of frustration, unawareness of the disorder, negative evaluation, indecisiveness, and loss of focus and loss of memory. Another major category was physical illnesses with the subcategories of physical discomfort, sleep problems, appetite disturbance, facial changes, sexual dysfunction, and medical conditions. The final category was failure in life, which had failure in personal affairs, jeopardized interpersonal relations, and unstable work life as subcategories. These findings provide a base for further research in this area. They also have clinical relevance for health care providers working with patients with MDD. Related cultural issues also are discussed.

Association between cognitive deficits and suicidal ideation in patients with major depressive disorder

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Pu, Shenghong; Setoyama, Shiori; Noda, Takamasa

2017-01-01

The role of cognitive function in suicidal ideation in patients with major depressive disorder (MDD) has not been adequately explored. This research sought to measure the relationship between suicidal ideation and cognitive function. Therefore, in this study, the association between cognitive function and suicidal ideation in patients with MDD was assessed. Cognitive function was evaluated in 233 patients with MDD using the Japanese version of the Brief Assessment of Cognition in Schizophreni...

Plain Radiography May Be Safely Omitted for Selected Major Trauma Patients Undergoing Whole Body CT: Database Study

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Sarah Hudson

2012-01-01

Full Text Available Introduction. Whole body CT is being used increasingly in the primary survey of major trauma patients. We evaluated whether omitting plain films of the chest and pelvis in the primary survey was safe. We compared the probability of survival of patients and time to CT who had plain X-rays to those who did not. Method. We performed a database study on major trauma patients admitted between 2008 and 2010 using data from Trauma, Audit and Research Network (TARN and our PACS system. We included adult major trauma patients who has an ISS of greater than 15 and underwent whole body CT. Results. 245 patients were included in the study. 44 (17.9% did not undergo plain films. The median time to whole body CT from the time of admission was longer (47 minutes in patients having plain films, than those who did not have plain films performed (30 minutes, P<0.005. Mortality was increased in the group who received plain films, 9.5% compared to 4.5%, but this was not statistically significant (P=0.77. Conclusion. We conclude that plain films may be safely omitted during the primary survey of selected major trauma patients.

The Relationship between Thyroid Function and Recent History of Suicide Attempt in Patients with Major Depression

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Abbas Eshraghi

2016-03-01

Full Text Available Background and Objectives: Todays, evaluation of the relationship between thyroid function and some psychiatric diseases have been identified. However, studies on the relationship between thyroid function and suicide attempt are limited. The present study was carried out with the aim of evaluating thyroid function in patients attempting suicide. Methods: In this descriptive analytical study during the years 2011 and 2012, 88 patients with major depression and recent history of suicide attemp and 89 patients with major depression without history of recent suicide, who were hospitalized in the psychiatric ward of Hazrat Rasoul Akram Hospital in Tehran, were included in the study. The studied variables in this research included demographic variables, such as age, gender as well as clinical findings, such as thyroid function tests, including TSH, T3, T4. thyroid function tests were requested for patients when hospitalized with a diagnosis of major depression during the years 2011 and 2012, that these values were extracted from the patients’ medical records. Results: The two groups were matched in terms of age and gender. In patients with recent suicide attempt, 5 (5.6% cases of clinical hypothyroidism and 6 (6.8% cases of subclinical hypothyroidism, were reported. In the major depression patients without recent suicide attempt, there were 3 (3.3% cases of clinical hypothyroidism and 6 (6.7% cases of subclinical hypothyroidism, and the two groups had no significant difference in terms of the incidence of thyroid disease (p=0.75. Conclusion: According to the results of the present study, lower levels of T3 and T3 to T4 ratio can be one of the factors related to the recent history of suicide in patients with major depression.

Impaired social decision making in patients with major depressive disorder.

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Wang, Yun; Zhou, Yuan; Li, Shu; Wang, Peng; Wu, Guo-Wei; Liu, Zhe-Ning

2014-01-23

Abnormal decision-making processes have been observed in patients with major depressive disorder (MDD). However, it is unresolved whether MDD patients show abnormalities in decision making in a social interaction context, in which decisions have actual influences on both the self-interests of the decision makers per se and those of their partners. Using a well-studied ultimatum game (UG), which is frequently used to investigate social interaction behavior, we examined whether MDD can be associated with abnormalities in social decision-making behavior by comparing the acceptance rates of MDD patients (N = 14) with those of normal controls (N = 19). The acceptance rates of the patients were lower than those of the normal controls. Additionally, unfair proposals were accepted at similar rates from computer partners and human partners in the MDD patients, unlike the acceptance rates in the normal controls, who were able to discriminatively treat unfair proposals from computer partners and human partners. Depressed patients show abnormal decision-making behavior in a social interaction context. Several possible explanations, such as increased sensitivity to fairness, negative emotional state and disturbed affective cognition, have been proposed to account for the abnormal social decision-making behavior in patients with MDD. This aberrant social decision-making behavior may provide a new perspective in the search to find biomarkers for the diagnosis and prognosis of MDD.

Hypoparathyroidism and intracerebral calcification in patients with beta-thalassemia major

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Karimi, M. [Iran-Shiraz-Namazee Hospital, Namazee Square, Hematology Research Center, Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: karimim@sums.ac.ir; Rasekhi, A.R. [Iran-Shiraz-Namazee Hospital, Namazee Square, Imaging Research Center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: rasekhia@sums.ac.ir; Rasekh, M. [Iran-Shiraz-Namazee Hospital, Namazee Square, Department of Endocrinology and Metabolism, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: Rasekhm@sums.ac.ir; Nabavizadeh, S.A. [Iran-Shiraz-Namazee Hospital, Namazee Square, Imaging Research Center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: nabavia@gmail.com; Assadsangabi, R. [Iran-Shiraz-Namazee Hospital, Namazee Square, Imaging Research Center, Department of Radiology, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: assadsangabi@yahoo.com; Amirhakimi, G.H. [Iran-Shiraz-Namazee Hospital, Namazee Square, Department of Endocrinology and Metabolism, Shiraz University of Medical Sciences, Shiraz (Iran, Islamic Republic of)], E-mail: amirhakimig@sums.ac.ir

2009-06-15

Background: Hypoparathyroidism is one of the most important endocrine complications of thalassemia major. This study was conducted to evaluate the prevalence of intracerebral calcifications in patients with thalassemia with and without hypoparathyroidism. Methods: 47 beta-thalassemia patients with hypoparathyroidism underwent a brain CT scan to investigate the presence and extent of intracerebral calcification. 30 age- and sex-matched beta-thalassemic patients with normal parathyroid function who had undergone brain CT for headache, or some other minor neurologic problems were also enrolled in the study serving as controls. The amount of intracerebral calcification, hematologic parameters, and some clinical findings were compared between both groups. Results: Intracerebral calcification was present in 54.2% of beta-thalassemia patients with hypoparathyroidism. The most frequent sites of calcification were basal ganglia, and frontoparietal areas of the brain. Thalami, internal capsule, cerebellum and posterior fossa were other less frequently calcified regions of the brain. In contrast, there was no evidence of intracerebral calcifications in the 30 thalassemic patients with normal parathyroid function. There was not a statistically significant difference between serum ferritin concentrations in thalassemia patient with hypoparathyroidism and those with normal parathyroid function (2781 vs. 2178, P > 0.05). Conclusion: Intracranial calcification is a common finding in thalassemia patients with hypoparathyroidism, it can be extensive and involves most regions of the brain.

Hypoparathyroidism and intracerebral calcification in patients with beta-thalassemia major

International Nuclear Information System (INIS)

Karimi, M.; Rasekhi, A.R.; Rasekh, M.; Nabavizadeh, S.A.; Assadsangabi, R.; Amirhakimi, G.H.

2009-01-01

Background: Hypoparathyroidism is one of the most important endocrine complications of thalassemia major. This study was conducted to evaluate the prevalence of intracerebral calcifications in patients with thalassemia with and without hypoparathyroidism. Methods: 47 beta-thalassemia patients with hypoparathyroidism underwent a brain CT scan to investigate the presence and extent of intracerebral calcification. 30 age- and sex-matched beta-thalassemic patients with normal parathyroid function who had undergone brain CT for headache, or some other minor neurologic problems were also enrolled in the study serving as controls. The amount of intracerebral calcification, hematologic parameters, and some clinical findings were compared between both groups. Results: Intracerebral calcification was present in 54.2% of beta-thalassemia patients with hypoparathyroidism. The most frequent sites of calcification were basal ganglia, and frontoparietal areas of the brain. Thalami, internal capsule, cerebellum and posterior fossa were other less frequently calcified regions of the brain. In contrast, there was no evidence of intracerebral calcifications in the 30 thalassemic patients with normal parathyroid function. There was not a statistically significant difference between serum ferritin concentrations in thalassemia patient with hypoparathyroidism and those with normal parathyroid function (2781 vs. 2178, P > 0.05). Conclusion: Intracranial calcification is a common finding in thalassemia patients with hypoparathyroidism, it can be extensive and involves most regions of the brain.

Ipsilateral atrophy of the psoas major muscle in patients with lumbar disc herniation

International Nuclear Information System (INIS)

Makino, Takahiro; Hosono, Noboru; Mukai, Yoshihiro; Miwa, Toshitada; Fuji, Takeshi

2009-01-01

We measured the cross-sectional area (CSA) of the psoas major muscles of 48 male patients under 50 years of age with unilateral sciatica caused by a single-level lumbar disc herniation. Patients who had multi-level disc lesions, lumbar canal stenosis, spondylolisthesis, scoliosis>5deg, or a history of lumbar surgery or hip joint disease were excluded. Mean age at surgery was 33 years old. Two orthopedic surgeons measured the CSA independently and blindly on magnetic resonance images in which the spinal canal had been blacked out. The CSA ratio (pain-positive side/pain-negative side) was 0.99 at L3/4, 0.98 at L4/5, and 1.00 at L5/S. There was a statistically significant difference between the CSA of the psoas major muscle on the painful side and the unaffected side at L4/5 (p=0.02). There was no correlation between the CSA ratio and the angle in the straight leg raising test, the duration of symptoms, or the size of the disc herniation. The atrophy of the psoas major muscle observed on the pain-positive side in lumbar disc herniation patients may be attributable to disuse of the affected leg. (author)

Pulsed and Tissue Doppler Echocardiographic Changes in Patients with Thalassemia Major

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Taysir S. Garadah

2010-01-01

Full Text Available Background Doppler echocardiographic studies of left ventricle (LV systolic and diastolic function in patients with β-Thalassemia Major (β-TM had shown different patterns of systolic and diastolic dysfunction. Aim This cross-sectional study was designed to study the LV systolic and diastolic function in patients with β-TM using Pulsed Doppler (PD and Tissue Doppler (TD echocardiography. Methods All patients were evaluated clinically and by echocardiography, The study included patients with β-TM (n = 38, age 15.7 ± 8.9 years compared with an age-matched control group (n = 38, age 15.9 ± 8.9 years. The pulse Doppler indices were normalized for age and heart rate. Results Compared with control patients, M-Mode showed that patients with β-TM have thicker LV septal wall index (0.659 ± 0.23 vs. 0.446 ± 0.219 cm, P ≤ 0.001, posterior wall index (0.659 ± 0.235 vs. 0.437 ± 0.214 cm, P ≤ 0.01, and larger LVEDD index is (3.99 ± 0.48 vs. 2.170 ± 0.57 mm. P = 0.035. Pulsed Doppler showed high LV trans-mitral E wave velocity (70.818 ± 10.139 vs. 57.532 ± 10.139, p = 0.027 and E/A ratio (1.54 vs. 1.23, P ≤ 0.01. The duration of Deceleration time (DT and isovolumic relaxation time (IVRT were significantly shorter in patients with β-TM (150.234 ± 20.0.23 vs. 167.123 ± 19.143 msec, P ≤ 0.01 and (60.647 ± 6.77 vs. 75.474 ± 5.83 msec, P ≤ 0.001, respectively. The ratio of transmitral E wave velocity to the tissue Doppler E wave at the basal septal mitral annulus E/Em – was significantly higher in β-TM group (14.024 ± 2.29 vs. 12.132 ± 1.82, P ≤ 0.01. The Tissue Doppler systolic velocity (Sm and the early diastolic velocity (Em were significantly lower in β-TM group compared to control (4.31 ± 1.2 cm/s vs. 6.95 ± 2.1, P ≤ 0.01 and 4.31 ± 2.7 cm/s vs. 5.82 ± 2.5, P ≤ 0.01 respectively. The tricuspid valve velocity was significantly higher than controls (2.993 ± 0.569 vs. 1.93 ± 0.471 m/sec, respectively, P ≤ 0

Slow sleep spindle and procedural memory consolidation in patients with major depressive disorder

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Nishida M

2016-01-01

Full Text Available Masaki Nishida,1 Yusaku Nakashima,2 Toru Nishikawa11Department of Psychiatry and Behavioral Sciences, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Yushima, Bunkyo, 2Medical Technology Research Laboratory, Research and Development Division, Medical Business Unit, Sony Corporation, Tokyo, JapanIntroduction: Evidence has accumulated, which indicates that, in healthy individuals, sleep enhances procedural memory consolidation, and that sleep spindle activity modulates this process. However, whether sleep-dependent procedural memory consolidation occurs in patients medicated for major depressive disorder remains unclear, as are the pharmacological and physiological mechanisms that underlie this process.Methods: Healthy control participants (n=17 and patients medicated for major depressive disorder (n=11 were recruited and subjected to a finger-tapping motor sequence test (MST; nondominant hand paradigm to compare the averaged scores of different learning phases (presleep, postsleep, and overnight improvement. Participants' brain activity was recorded during sleep with 16 electroencephalography channels (between MSTs. Sleep scoring and frequency analyses were performed on the electroencephalography data. Additionally, we evaluated sleep spindle activity, which divided the spindles into fast-frequency spindle activity (12.5–16 Hz and slow-frequency spindle activity (10.5–12.5 Hz.Result: Sleep-dependent motor memory consolidation in patients with depression was impaired in comparison with that in control participants. In patients with depression, age correlated negatively with overnight improvement. The duration of slow-wave sleep correlated with the magnitude of motor memory consolidation in patients with depression, but not in healthy controls. Slow-frequency spindle activity was associated with reduction in the magnitude of motor memory consolidation in both groups.Conclusion: Because the changes in slow

Male Patient Visits to the Emergency Department Decline During the Play of Major Sporting Events

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Jerrard, David A

2009-05-01

Full Text Available OBJECTIVES: To study whether emergency department (ED visits by male patients wane simultaneously with the play of scheduled professional and college sports events.METHODS: Retrospective cohort analysis looked at ED male patient registration rates during a time block lasting from two hours before, during, and two hours after the play of professional football games (Monday night, Sundays, post-season play, major league baseball, and a Division I college football and basketball team, respectively. These registration rates were compared to rates at similar times on similar days of the week during the year devoid of a major sporting contest. Games were assumed to have a play time of three hours. Data was collected from April 2000 through March 2003 at an urban academic ED seeing 33,000 male patients above the age of 18 years annually.RESULTS: A total of 782 games were identified and used for purposes of the study. Professional football game dates had a mean of 17.9 males (95% confidence interval [CI] 17.4-18.4 registering vs. 26.8 males (95% CI 25.9-27.6 on non-game days. A registration rate for major league baseball was 18.4 patients (95% CI 17.6-18.4. The mean for registration on comparable non-game days was 23.9 patients (95% CI 22.8-24.3. For the regional Division I college football team, the mean number of patients registering on game days and non-game days was 21.7 (95% CI 20.9-22.4 and 23.4 (95% CI 22.9-23.7, respectively. Division I college basketball play for game and non-game days had mean rates of registration of 14.5 (95% CI 13.9-15.1 and 15.5 (95% CI 15.1-15.9 patients, respectively. For all sports dates collectively, a comparison of two means yielded a mean of 18.2 patients (95% CI 17.4-18.8 registering during the study hours on game days vs. 23.3 patients (95% CI 22.0-23.7 on non-game days. The mean difference was 5.1 patients (95% CI 3.7 to 7.0 with p < .000074.CONCLUSION: Male patient visits to the ED decline during major sporting

Prospective mental imagery in patients with major depressive disorder or anxiety disorders

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Morina, N.; Deeprose, C.; Pusowski, C.; Schmid, M.; Holmes, E.A.

2011-01-01

Prospective negative cognitions are suggested to play an important role in maintaining anxiety disorders and major depressive disorder (MDD). However, little is known about positive prospective mental imagery. This study investigated differences in prospective mental imagery among 27 patients with

Thoracic epidural analgesia reduces myocardial injury in ischemic patients undergoing major abdominal cancer surgery

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Mohamad MF

2017-04-01

Full Text Available Mohamad Farouk Mohamad,1 Montaser A Mohammad,1 Diab F Hetta,1 Eman Hasan Ahmed,2 Ahmed A Obiedallah,3 Alaa Ali M Elzohry1 1Department of Anesthesia, ICU and Pain Relief, 2Department of Clinical Pathology, South Egypt Cancer Institute, 3Department of Internal Medicine, Faculty of Medicine, Assiut University, Arab Republic of Egypt Background and objectives: Major abdominal cancer surgeries are associated with significant perioperative mortality and morbidity due to myocardial ischemia and infarction. This study examined the effect of perioperative patient controlled epidural analgesia (PCEA on occurrence of ischemic cardiac injury in ischemic patients undergoing major abdominal cancer surgery.Patients and methods: One hundred and twenty patients (American Society of Anesthesiologists grade II and III of either sex were scheduled for elective upper gastrointestinal cancer surgeries. Patients were allocated randomly into two groups (60 patients each to receive, besides general anesthesia: continuous intra and postoperative intravenous (IV infusion with fentanyl for 72 h postoperatively (patient controlled intravenous analgesia [PCIA] group or continuous intra and postoperative epidural infusion with bupivacaine 0.125% and fentanyl (PCEA group for 72 h postoperatively. Perioperative hemodynamics were recorded. Postoperative pain was assessed over 72 h using visual analog scale (VAS. All patients were screened for occurrence of myocardial injury (MI by electrocardiography, echocardiography, and cardiac troponin I serum level. Other postoperative complications as arrhythmia, deep venous thrombosis (DVT, pulmonary embolism, pneumonia, and death were recorded.Results: There was a significant reduction in overall adverse cardiac events (myocardial injury, arrhythmias, angina, heart failure and nonfatal cardiac arrest in PCEA group in comparison to PCIA group. Also, there was a significant reduction in dynamic VAS pain score in group PCEA in comparison

The Impact of Migrations on the Health Services for Rare Diseases in Europe: The Example of Haemoglobin Disorders

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Angastiniotis, Michalis; Vives Corrons, Joan-Lluis; Soteriades, Elpidoforos S.; Eleftheriou, Androulla

2013-01-01

Migration from different parts of the world to several European countries leads to the introduction of haemoglobinopathy genes into the population, which creates several demanding needs for prevention and treatment services for Hb disorders. In this paper we examined the degree to which European health services have responded to such challenges and in particular to health services necessary to address the needs of patients with thalassaemia and sickle cell disease (SCD). Information on available services was obtained from international organizations, collaborated European project, and the Thalassaemia International Federation (TIF) Databases, which include information from published surveys, registries, field trips, and delegation visits to countries and regions by expert advisors, local associations, and other collaborators' reports. Results show that countries with traditional strong prevention and treatment programs are well prepared to face the above challenges, while others are urgently needed to address these problems in a systematic way. The Thalassaemia International Federation (TIF) is committed to monitor the progress, raise awareness, and support the promotion of more immigrant-oriented health policies to ensure their integration in society and their access to appropriate, adequate, and timely health services. PMID:23576907

The Impact of Migrations on the Health Services for Rare Diseases in Europe: The Example of Haemoglobin Disorders

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Michalis Angastiniotis

2013-01-01

Full Text Available Migration from different parts of the world to several European countries leads to the introduction of haemoglobinopathy genes into the population, which creates several demanding needs for prevention and treatment services for Hb disorders. In this paper we examined the degree to which European health services have responded to such challenges and in particular to health services necessary to address the needs of patients with thalassaemia and sickle cell disease (SCD. Information on available services was obtained from international organizations, collaborated European project, and the Thalassaemia International Federation (TIF Databases, which include information from published surveys, registries, field trips, and delegation visits to countries and regions by expert advisors, local associations, and other collaborators’ reports. Results show that countries with traditional strong prevention and treatment programs are well prepared to face the above challenges, while others are urgently needed to address these problems in a systematic way. The Thalassaemia International Federation (TIF is committed to monitor the progress, raise awareness, and support the promotion of more immigrant-oriented health policies to ensure their integration in society and their access to appropriate, adequate, and timely health services.

Sleep disturbances in patients with major depressive disorder: incongruence between sleep log and actigraphy.

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Kung, Pei-Ying; Chou, Kuei-Ru; Lin, Kuan-Chia; Hsu, Hsin-Wei; Chung, Min-Huey

2015-02-01

Depression has become a severe global health problem, and sleeping difficulties are typically associated with depression. The purpose of this study was to investigate the relationships among subjective sleep quality, objective sleep quality, and the sleep hygiene practices of hospitalized patients with major depressive disorder. Daily sleep logs and actigraphy were used to obtain subjective and objective sleep data. Thirty patients were recruited from a regional teaching hospital in Taipei and completed the Hamilton Rating Scale for Depression and the Sleep Hygiene Practice Scale. Significant differences were found between subjective and objective sleep data in patients with major depressive disorder (MDD). For patients with more severe depression, subjective measurements obtained using sleep logs, such as total sleep time and sleep efficiency, were significantly lower than those obtained using actigraphy by controlling for demographics. The results regarding the differences between subjective and objective sleep data can be a reference for care providers when comforting depression patients who complain of sleep disturbance. Copyright © 2014 Elsevier Inc. All rights reserved.

Mixed chimerism following hematopoietic stem cell transplantation in pediatric thalassemia major patients: a single center experience

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Elif Ünal İnce

2010-03-01

Full Text Available Objective: Stable mixed chimerism (MC may result in cure for thalassemia major patients following hematopoietic stem cell transplantation (HSCT, but rejection can occur. Twenty-eight HSCTs for thalassemia major were reviewed retrospectively to evaluate the clinical course of MC with possible risk factors and predictors of outcome, with a median follow-up of 1669 days (811-3576 days. Materials and Methods: Chimerism was detected by fluorescence in situ hybridization (FISH or multiplex polymerase chain reaction depending on the sex match between the donor and the recipient. Results: Primary rejection, stable MC and full donor chimerism was detected in 3.6%, 17.8% and 78.6% of patients, respectively. Clinically, 4/5 patients with stable MC had thalassemia trait with donor chimerism as low as 14%. One patient was started on pRBC transfusions at 2.5 years postHSCT. Conclusion: Stable MC can result in cure for thalassemia major patients. The clinical picture remains as the best guide for intervention until a more reliable predictor is available.

Minor Bleeds Alert for Subsequent Major Bleeding in Patients Using Vitamin K Antagonists.

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Veeger , Nic J.G.M.; Piersma-Wichers , Margriet; Meijer , Karina; Hillege , Hans L.

2011-01-01

Abstract Vitamin K antagonists (VKA) have shown to be effective in primary and secondary prevention of thromboembolism, but the associated risk of bleeding is an important limitation. The majority of the bleeds are clinically mild. In this study, we assessed whether these minor bleeds are associated with major bleeding, when controlling for other important risk indicators, including the achieved quality of anticoagulation. For this, 5898 patients of a specialised anticoagulation cl...

Outcome of patients with major depressive disorder after serious suicide attempt.

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Suominen, Kirsi; Haukka, Jari; Valtonen, Hanna M; Lönnqvist, Jouko

2009-10-01

To investigate the outcome of subjects with major depressive disorder after serious suicide attempt and to examine the effect of psychotic symptoms on their outcome. The study population included all individuals aged 16 years or older in Finland who were hospitalized with ICD-10 diagnoses of major depressive disorder and attempted suicide from 1996 to 2003 (N = 1,820). The main outcome measures were completed suicides, overall mortality, and repeated suicide attempts during drug treatment versus no treatment. During the 4-year follow-up period, 13% of patients died, 6% completed suicide, and 31% made a repeat suicide attempt. Subjects with major depression with psychotic features completed suicide more often than subjects without psychotic features during the follow-up (hazard ratio [HR] 3.32; 95% CI, 1.95 - 5.67). Antidepressant treatment reduced all-cause mortality by 24% (HR 0.74; 95% CI, 0.56 - 0.97) but did not reduce suicide mortality (HR 1.06; 95% CI, 0.71 - 1.58). Psychotic symptoms during major depressive episode increase the risk of completed suicide after serious suicide attempt. The quality of treatment for major depression with psychotic features after attempted suicide should be improved to prevent suicide. Copyright 2009 Physicians Postgraduate Press, Inc.

A comparison of heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major.

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Amoozgar, Hamid; Zeighami, Samaneh; Haghpanah, Sezaneh; Karimi, Mehran

2017-01-01

The goal of this study was to compare heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major. In this cross-sectional study, 60 patients with beta thalassemia major and 60 patients with beta thalassemia intermedia who had clinically no symptoms of arrhythmia and clinically normal heart function were evaluated using 24-hour ambulatory electrocardiogram monitoring and echocardiography. For data analysis SPSS ver.20 software was used. A P-value of less than 0.05 was considered statistically significant. The mean age of the beta thalassemia intermedia patients was 24.18 ± 7.9 years and the mean age in beta thalassemia major was 24.38 ± 7.7 years (P>0.05). Premature atrial contractions (PACs) were observed in 14 (23.3%) patients with beta thalassemia intermedia and in 22 (36.6%) beta thalassemia major patients. Premature ventricular contractions (PVCs) were detected in 8 (13.3%) patients in the beta thalassemia intermediate group and 16 (26.6) patients in the beta thalassemia major group, respectively. The left ventricular diastolic dimension, end-diastolic volume, and stroke volume were significantly higher in beta thalassemia intermedia group (Pintermedia group. Both atrial and ventricular arrhythmias were more common in the beta thalassemia major group. Higher end-diastolic volume and stroke volume were detected in the beta thalassemia intermedia group. Pulmonary acceleration time was lower in the beta thalassemia intermedia group, which can be an indicator of higher pulmonary pressure.

Cognitive–behavioral group therapy is an effective treatment for major depression in hemodialysis patients

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Duarte, Priscila Silveira; Miyazaki, Maria Cristina; Blay, Sergio Luís; Sesso, Ricardo

2009-01-01

Depression is an important target of psychological assessment in patients with end-stage renal disease because it predicts their morbidity, mortality, and quality of life. We assessed the effectiveness of cognitive-behavioral therapy in chronic hemodialysis patients diagnosed with major depression by the Mini International Neuropsychiatric Interview (MINI). in a randomized trial conducted in Brazil, an intervention group of 41 patients was given 12 weekly sessions of cognitive-behavioral grou...

Predictors of the Onset of Manic Symptoms and a (Hypo)Manic Episode in Patients with Major Depressive Disorder

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Boschloo, Lynn; Spijker, Annet T.; Hoencamp, Erik; Kupka, Ralph; Nolen, Willem A.; Schoevers, Robert A.; Penninx, Brenda W. J. H.

2014-01-01

Objective: One third of patients with a major depressive episode also experience manic symptoms or, even, a (hypo) manic episode. Retrospective studies on the temporal sequencing of symptomatology suggest that the majority of these patients report depressive symptoms before the onset of manic

Termination of pregnancy for fetal anomaly in a Tunisian population

African Journals Online (AJOL)

Chromosomal anomalies included 10 cases of trisomy 21. (62.5%), 2 cases of trisomy 18 (12.5%), 3 cases of trisomy 13 (18.7%) and 1 case of a deletion of the short arm of chromosome 18. All fetal anomalies were suspected by antenatal ultrasound, except for a case of β-thalassaemia major. The first antenatal ultrasound ...

Patients' health or company profits? The commercialisation of academic research.

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Olivieri, Nancy F

2003-01-01

This paper is a personal account of the events associated with the author's work at the University of Toronto's Hospital for Sick Children on a drug, deferiprone, for the treatment of thalassaemia. Trials of the drug were sponsored by the Canadian Medical Research Council and a drug company which would have been able, had the trials been successful, to seek regulatory approval to market the drug. When evidence emerged that deferiprone might be inadequately effective in a substantial proportion of patients, the drug company issued legal threats when the author proposed informing her patients and the scientific community. Until protests were made by international authorities in her field of research, the hospital and university did not adequately support the author's academic freedom and responsibilities as a medical practitioner. It is argued that underlying cause of this, and of other similar cases, is the political philosophy which is driving the commercialisation of universities and bringing about the deregulation of drug approval procedures. Together these changes constitute a serious threat to the public good.

Prospects after Major Trauma

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Holtslag, H.R.

2007-01-01

Introduction. After patients survived major trauma, their prospects, in terms of the consequences for functioning, are uncertain, which may impact severely on patient, family and society. The studies in this thesis describes the long-term outcomes of severe injured patients after major trauma. In

Nurses' knowledge and attitudes regarding major immobility complications among bedridden patients: A prospective multicentre study.

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Li, Zhen; Zhou, Xinmei; Cao, Jing; Li, Zheng; Wan, Xia; Li, Jiaqian; Jiao, Jing; Liu, Ge; Liu, Ying; Li, Fangfang; Song, Baoyun; Jin, Jingfen; Liu, Yilan; Wen, Xianxiu; Cheng, Shouzhen; Wu, Xinjuan

2018-05-01

To gain insight into nurses' knowledge and attitudes regarding major immobility complications (pressure ulcers, pneumonia, deep vein thrombosis and urinary tract infections) and explore the correlation of nurses' knowledge and attitudes with the incidence of these complications. Immobility complications have adverse consequences, and effective management requires appropriate knowledge, attitudes and skills. Evidence about nurses' knowledge and attitudes regarding immobility complications is lacking. Cross-sectional study. A total of 3,903 nurses and 21,333 bedridden patients from 25 hospitals in China were surveyed. Nurses' knowledge and attitudes regarding major immobility complications were assessed using researcher-developed questionnaires. The content validity, reliability and internal consistency of the questionnaires were validated through expert review and a pilot study. The incidence of major immobility complications among bedridden patients from selected wards was surveyed by trained investigators. Correlations between knowledge, attitudes and the incidence of major immobility complications were evaluated with multilevel regression models. Mean knowledge scores were 64.07% for pressure ulcers, 72.92% for deep vein thrombosis, 76.54% for pneumonia and 83.30% for urinary tract infections. Mean attitude scores for these complications were 86.25%, 84.31%, 85.00% and 84.53%, respectively. Knowledge and attitude scores were significantly higher among nurses with older age, longer employment duration, higher education level, previous training experience and those working in tertiary hospitals or critical care units. Nurses' knowledge about pressure ulcers was negatively related to the incidence of pressure ulcers, and attitude towards pneumonia was negatively correlated with the incidence of pneumonia. Clinical nurses have relatively positive attitudes but inadequate knowledge regarding major immobility complications. Improved knowledge and attitudes regarding

Cognitive Effects of Electroconvulsive Therapy in Patients with Major Depressive, Bipolar and Schizophrenia Disorders

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N Fouladi

2011-10-01

Full Text Available Background & Aim: Electroconvulsive therapy (ECT is a highly effective treatment for affective and schizophrenic disorders. The main objective of this study was to examine the cognitive effects of ECT in patients with major depressive, bipolar and schizophrenia disorders. Methods: In this study we administered a battery of cognitive tasks on 90 patients with major depressive, bipolar and schizophrenia disorders, one day before and after the termination of ECT. The effects were measured by a set of computerized cognitive tests including: auditory reaction time, visual reaction time, verbal memory, Benton visual memory, Wisconsin card sort and motor function. The collected data were analyzed using One-way ANOVA and dependent t-test. Results: The results showed that depressive patients had poorer verbal memory and motor function after the termination of ECT compared to pretest, but their executive function was improved (p<0.05. After the termination of ECT the verbal and visual memory and executive function was significantly improved in patients with bipolar and schizophrenia disorders but their motor function was significantly reduced (p<0.05. Conclusion: Results of this study showed improvement for most cognitive functions in patients after electroconvulsive therapy. Findings of this study may help patients and their families to overcome their fear of electroconvulsive therapy. The results also can aware patients regarding the cognitive effects of electroconvulsive therapy.

Posterior reversible encephalopathy syndrome: Some novel ...

African Journals Online (AJOL)

Two cases occurred following cerebral anoxia due to accidental strangulation and near-drowning, respectively. The third patient, a child known to have E-β thalassaemia, presented with transient encephalopathy following blood transfusion but involving the anterior brain rather than the posterior part classically described in ...

Adipocytokine concentrations in children with different types of beta-thalassemia.

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Enli, Yaşar; Balci, Yasemin I; Gönen, Cafer; Uzun, Ebru; Polat, Aziz

2014-06-01

Beta-thalassemia is an inherited blood disorder. It results from the impaired production of β-globin chains, leading to a relative excess of alpha-globin chains. Clinical severity separates this disease into three main subtypes: β- thalassemia major, β-thalassemia intermedia and β-thalassemia minor, the former two being clinically more significant. Inflammatory processes may play an important role in some of the complications of thalassemia. Adipose tissue is one of the most important endocrine and secretory organs that release adipocytokines like adiponectin, resistin and visfatin. The aim of our study was to analyze adipocytokine concentrations (adiponectin, resistin and visfatin) in different types of β-thalassemia patients and determine any possible correlations with disease severity. We recruited 29 patients who were transfusion-dependent β-thalassemia-major patients, 17 patients with β-thalassemia intermedia, 30 β-thalassemia minor patients. The control group consisted of 30 healthy children. Anthropometric measurements, complete blood count, biochemical parameters, serum concentrations of adiponectin, resistin, visfatin were performed for all subjects. Resistin and visfatin concentrations were significantly higher in β-thalassemia minor patients than in controls. Adiponetin, resistin and visfatin concentrations were significantly higher in both β-thalassemia intermedia and major patients than in controls. The concentrations of adiponectin, resistin and visfatin were significantly higher in both β-thalassemia intermedia and major patients than in β-thalassemia minor patients. There was no significant difference between β-thalassemia intermedia and β-thalassemia major patients for adipocytokines concentrations. We speculate that these adipocytokines may play a role in the development of complications in β-thalassaemia.

Levels of Beta-2 Microglobulin and Cystatin C in Beta Thalassemia Major Patients

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Ayse Kacar

2014-03-01

Full Text Available Aim: Thalassemia is accepted to be the most common genetic disease in the world. This study was performed to establish whether there was a glomerular renal damage, which was usually a less mentioned subject in patients with Beta Thalassemia Major, and to compare urea, creatinine and creatinine clearance with early indicators of kidney damage as Cystatin-C and %u03B2-2 microglobulin as on determining the glomerular damage. Material and Method: This study was prospectively performed in patients, who were regularly followed in the children hematology outpatient clinic with a diagnosis of Beta Thalassemia Major. Results: There was no statistically significant difference between urea and levels of creatinine clearance and Cystatin-C. There was a statistically negative relationship between creatinine and creatinine clearance at an advanced level as 53.7% (p: 0.002, p

Dark blood versus bright blood T2* acquisition in cardiovascular magnetic resonance (CMR) for thalassaemia major (TM) patients: Evaluation of feasibility, reproducibility and image quality

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Liguori, Carlo, E-mail: c.liguori@unicampus.it [Department of Diagnostic Imaging, Campus Bio Medico University, via Alvaro del Portillo 200, 00128 Rome (Italy); Di Giampietro, Ilenia; Pitocco, Francesca; De Vivo, Aldo Eros [Department of Diagnostic Imaging, Campus Bio Medico University, via Alvaro del Portillo 200, 00128 Rome (Italy); Schena, Emiliano [Unit of Measurements and Biomedical Instrumentation, Campus Bio Medico University, via Alvaro del Portillo 200, 00128 Rome (Italy); Mortato, Luca [Department of Diagnostic Imaging, Campus Bio Medico University, via Alvaro del Portillo 200, 00128 Rome (Italy); Pirro, Federica [Department of Biomaging and Radiological Sciences, Catholic University of Sacred Herart, Largo A. Gemelli 1, 00135 Rome (Italy); Cianciulli, Paolo [Thalassemia Unit, Ospedale Sant Eugenio, Piazzale dell’Umanesimo 10, 00143 Rome (Italy); Zobel, Bruno Beomonte [Department of Diagnostic Imaging, Campus Bio Medico University, via Alvaro del Portillo 200, 00128 Rome (Italy)

2014-01-15

Objectives: To compare the effectiveness of dark blood (DB) versus bright blood (BB) sequences. To assess the intra and inter-observer variability and inter-study reproducibility between BB versus DB. To evaluate image quality level in the two sequences. Methods: In a setting of 138 patients we performed CMR using cardiac gated Gradient-multiecho single breath-hold BB and DB sequences in the middle ventricular septum. Each acquisition was repeated during the same exam. Truncation method was used to account for background noise. Image quality (IQ) was assessed using a 5 point grading scale and image analysis was conducted by 2 experienced observers. Results: Compared with the conventional BB acquisition, the coefficient of correlation and significance of the DB technique was superior for intra-observer reproducibility (p < 0.001), inter-observer reproducibility (p < 0.001) and inter-study reproducibility (p < 0.001). The variability is also lower for DB sequences for T2* values <14 ms. Assessment of artifacts showed a superior score for DB versus BB scans (4 versus 3, p < 0.001). Conclusions: Improvement in terms of inter observer and inter study variability using DB sequences was obtained. The greatest disparity between them was seen in inter-study reproducibility and higher IQ in DB was seen. Study demonstrates better performance of DB imaging compared to BB in presence of comparable effectiveness.

Propofol clearance and volume of distribution are increased in patients with major burns.

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Han, Tae-Hyung; Greenblatt, David J; Martyn, J A Jeevendra

2009-07-01

Propofol pharmacokinetics were examined in 17 adults with major burns during the hyperdynamic convalescent phase. Eighteen nonburned surgical patients served as controls. After a 2-mg/kg intravenous dose of propofol, blood samples were collected at multiple time points. Noncompartmental methods were used to calculate the pharmacokinetic parameters. The following indices were higher in burns than controls: propofol clearance (64+/-17 vs 29+/-4 mL/kg/min, Pclearance of propofol in burned patients may imply that these patients require higher doses or infusion rates of propofol to attain a target plasma concentration or pharmacodynamic effect.

Assessment of online patient education materials from major ophthalmologic associations.

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Huang, Grace; Fang, Christina H; Agarwal, Nitin; Bhagat, Neelakshi; Eloy, Jean Anderson; Langer, Paul D

2015-04-01

Patients are increasingly using the Internet to supplement finding medical information, which can be complex and requires a high level of reading comprehension. Online ophthalmologic materials from major ophthalmologic associations should be written at an appropriate reading level. To assess ophthalmologic online patient education materials (PEMs) on ophthalmologic association websites and to determine whether they are above the reading level recommended by the American Medical Association and National Institutes of Health. Descriptive and correlational design. Patient education materials from major ophthalmology websites were downloaded from June 1, 2014, through June 30, 2014, and assessed for level of readability using 10 scales. The Flesch Reading Ease test, Flesch-Kincaid Grade Level, Simple Measure of Gobbledygook test, Coleman-Liau Index, Gunning Fog Index, New Fog Count, New Dale-Chall Readability Formula, FORCAST scale, Raygor Readability Estimate Graph, and Fry Readability Graph were used. Text from each article was pasted into Microsoft Word and analyzed using the software Readability Studio professional edition version 2012.1 for Windows. Flesch Reading Ease score, Flesch-Kincaid Grade Level, Simple Measure of Gobbledygook grade, Coleman-Liau Index score, Gunning Fog Index score, New Fog Count, New Dale-Chall Readability Formula score, FORCAST score, Raygor Readability Estimate Graph score, and Fry Readability Graph score. Three hundred thirty-nine online PEMs were assessed. The mean Flesch Reading Ease score was 40.7 (range, 17.0-51.0), which correlates with a difficult level of reading. The mean readability grade levels ranged as follows: 10.4 to 12.6 for the Flesch-Kincaid Grade Level; 12.9 to 17.7 for the Simple Measure of Gobbledygook test; 11.4 to 15.8 for the Coleman-Liau Index; 12.4 to 18.7 for the Gunning Fog Index; 8.2 to 16.0 for the New Fog Count; 11.2 to 16.0 for the New Dale-Chall Readability Formula; 10.9 to 12.5 for the FORCAST scale; 11

Major depressive disorder and generalized anxiety disorder and response to treatment in hepatitis C patients in Egypt.

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MM, Bassiony; A, Yousef; U, Youssef; GM, Salah El-Deen; M, Abdelghani; H, Al-Gohari; E, Fouad; MM, El-Shafaey

2015-01-01

The aim of the study was to estimate the prevalence and associated correlates of major depressive disorder and generalized anxiety disorder in hepatitis C virus patients before and after treatment and to investigate the relationship between major depressive disorder and generalized anxiety disorder and treatment response. A total of 116 consecutive hepatitis C virus patients from hepatitis C virus treatment center in Zagazig city, Egypt, were included in the study and divided into treated group (N = 58) and untreated group (N = 58). All hepatitis C virus patients were screened for major depressive disorder and generalized anxiety disorder using hospital anxiety and depression scale, and those who screened positive were interviewed to confirm the diagnosis of major depressive disorder and generalized anxiety disorder using DSM-IV-TR diagnostic criteria. These measures were done at baseline and after 12 weeks of treatment or observation. At baseline, 3.5% and 12.1% of hepatitis C virus patients (treated group) had major depressive disorder and generalized anxiety disorder, respectively. After 12 weeks of treatment 37.9% of hepatitis C virus patients (treated group) had major depressive disorder and 46.6% had generalized anxiety disorder. There was a significant statistical difference between hospital anxiety and depression scale scores for depression (3.3 ± 2.3 vs. 6.4 ± 3.2, t = 9.6, p = 0.001) and for anxiety (4.6 ± 2.4 vs. 7.3 ± 3.0, t = 10.2, p = 0.001) before and after treatment. There was also significant statistical difference between treated group and untreated group regarding hospital anxiety and depression scale scores after treatment and observation (depression, treated group 6.4 ± 3.2 vs. untreated group 4.0 ± 2.4, t = 3.7, p = 0.001; anxiety, treated group 7.3 ± 3.0 vs. untreated group 4.5 ± 2.3, t = 4.4, p = 0.001). There was no association between major depressive disorder

Frequency of hepatitis B and hepatitis C in multi - transfused beta thalassemia major patients

International Nuclear Information System (INIS)

Iqbal, M.M.; Hassan, S.; Aziz, S.

2010-01-01

To determine the frequency of hepatitis B and C virus infection among children with beta thalassemia major registered at Military Hospital Rawalpindi. Children attending Thalassemia Centre Military Hospital Rawalpindi for regular blood transfusion were registered. They belonged to different ethnic groups and came from different parts of the country. Their demographic data was recorded, detailed history taken and physical examination was carried out. Their serum samples were tested for hepatitis B surface antigen and anti HCV antibody assay with third generation commercial ELISA method. During the study; 141 patients of beta thalassemia major were screened. Out of them 50 patients (35.5% ,95% confidence interval 27.8-43.5)w ere found hepatitis C virus antibody positive and 1 patient (0.7 %) hepatitis B surface antigen positive. One patient (0.7%) had both hepatitis B and C virus infection. Mean age of hepatitis C infected patients was 10.4+3.85y ears (range 2-16 years). Mean age of uninfected patients was 6.1 + 3.59 years. (p value 0.000) In addition, the results indicate that higher prevalence of anti-HCV was significantly associated with longer duration of transfusion (p value <0.003). In spite of the fact that screened blood is used for transfusions, still a large number of patients have been found infected with hepatitis C. Therefore more accurate techniques are required for screening of blood to prevent transfusion associated transmission. (author)

NUTRITIONAL ASSESSMENT IN PATIENTS PREDICTED TO MAJOR ABDOMINAL SURGERY AT THE GENERAL HOSPITAL CELJE

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Ernest Novak

2001-12-01

Full Text Available Background. Malnutrition has serious implications for recovery after surgery. Early detection of malnutrition with nutritional support minimizes postoperative complications. Nutritional assessment tools need to be simple and suitable for use in everyday practice. In our study we wanted to determine, how many patients might benefit from nutritional support.Methods. From April to August 1999 fifty consecutively admitted patients predicted to major abdominal surgery have been examined. We used Mini nutritional assessment (MNA, Buzby’s nutrition risk index (NRI, blood albumin level and weight loss in the last 3 months period prior to the examination, to assess nutritional status.Results. We examined 50 patients (27 males and 23 females, age 76.5 ± 16.5 and confirmed malnutrition in 40% of patients with MNA and serum albumin level. The increased risk for nutrition-associated complications was confirmed by NRI and weight loss in 44%.Conclusions. A confident diagnosis of malnutrition and increased risk for nutrition-associated complications can be established by using a combination of simple methods like MNA, NRI, weight loss and serum albumin level. Almost half of the patients admitted for major abdominal surgery in General hospital Celje suffer from malnutrition and they may benefit with early nutritional intervention.

Major depressive symptoms increase 3-year mortality rate in patients with mild dementia

DEFF Research Database (Denmark)

Petersen, Jindong Ding; Waldorff, Frans Boch; Siersma, Volkert Dirk

2017-01-01

Depression and dementia are commonly concurrent and are both associated with increased mortality among older people. However, little is known about whether home-dwelling patients newly diagnosed with mild dementia coexisting with depressive symptoms have excess mortality. We conducted a post hoc...... analysis based on data from the Danish Alzheimer's Intervention Study of 330 individuals who were diagnosed with mild dementia within the past 12 months. Thirty-four patients were identified with major depressive symptoms (MD-S) at baseline. During the 3-year follow-up period, 56 patients died, and, among...... mortality as compared to the patients without or with only few depressive symptoms. Our result revealed that depression is possibly associated with increased mortality in patients with mild dementia. Given that depression is treatable, screening for depression and treatment of depression can be important...

Sex differences in gut microbiota in patients with major depressive disorder.

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Chen, Jian-Jun; Zheng, Peng; Liu, Yi-Yun; Zhong, Xiao-Gang; Wang, Hai-Yang; Guo, Yu-Jie; Xie, Peng

2018-01-01

Our previous studies found that disturbances in gut microbiota might have a causative role in the onset of major depressive disorder (MDD). The aim of this study was to investigate whether there were sex differences in gut microbiota in patients with MDD. First-episode drug-naïve MDD patients and healthy controls were included. 16S rRNA gene sequences extracted from the fecal samples of the included subjects were analyzed. Principal-coordinate analysis and partial least squares-discriminant analysis were used to assess whether there were sex-specific gut microbiota. A random forest algorithm was used to identify the differential operational taxonomic units. Linear discriminant-analysis effect size was further used to identify the dominant sex-specific phylotypes responsible for the differences between MDD patients and healthy controls. In total, 57 and 74 differential operational taxonomic units responsible for separating female and male MDD patients from their healthy counterparts were identified. Compared with their healthy counterparts, increased Actinobacteria and decreased Bacteroidetes levels were found in female and male MDD patients, respectively. The most differentially abundant bacterial taxa in female and male MDD patients belonged to phyla Actinobacteria and Bacteroidia, respectively. Meanwhile, female and male MDD patients had different dominant phylotypes. These results demonstrated that there were sex differences in gut microbiota in patients with MDD. The suitability of Actinobacteria and Bacteroidia as the sex-specific biomarkers for diagnosing MDD should be further explored.

The Effect of Atropine on Post-ECT Bradycardia in Patients with Major Depressive Disorder

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Hassan Farashbandi

2014-08-01

Full Text Available Background: Electroconvulsive therapy (ECT is utilized for treatment of a range of psychiatric disorders including major depressive disorder (MDD. One of the major complications in using ECT is cardiovascular problems i.e., bradycardia. The present study was designed to investigate the effect of atropine on the pulse rate (PR of the patients under treatment with ECT. Materials and Methods: In this randomized clinical trial, 30 patients with diagnosis of MDD who received atropine before ECT treatment (control group were compared with 30 patients with the same diagnosis without receiving atropine (experimental group under ECT treatment. Both groups received ECT under the same term and condition. The PR of the patients were recorded 7 times (twice before anesthesia and ECT and 5 fixed one min intervals immediately after receiving ECT; for 10 sessions of treatment with ECT (3 times a week. The results were analyzed using repeated measure analysis of variance. The PR under 50 was the cut off point for differentiating the patients suffering from bradycardia and those without it. Results: Slight increment in PRs for experimental group (patient who did not receive atropine in contrast to control group were observed, but it did not reach a statistically significant level. The gender (male/female did not have different PR. The age of the patients and initial PR (regarded as co-variances did not show significant effect on PR for total sample. Conclusion: There seems to be not necessary to use atropine treatment for depressed patients receiving ECT.

Identification of major cardiovascular events in patients with diabetes using primary care data

NARCIS (Netherlands)

Pouwels, Koen Bernardus; Voorham, Jaco; Hak, Eelko; Denig, Petra

2016-01-01

Background: Routine primary care data are increasingly being used for evaluation and research purposes but there are concerns about the completeness and accuracy of diagnoses and events captured in such databases. We evaluated how well patients with major cardiovascular disease (CVD) can be

A 30-month study of patient complaints at a major Australian hospital.

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Anderson, K; Allan, D; Finucane, P

2001-12-01

Health practitioners often regard complaints about the quality of patient care in a negative light. However, complaints can indicate strategies to improve care. Therefore, an audit was undertaken of all formal complaints about patient care at a major Australian hospital over a 30-month period. The profile of complainants, the reasons for complaints, and the outcome were analysed. A total of 1308 complaints, concerning the care of 1267 patients, were received. The complaint rate was 1.12 per 1000 occasions of service. In all, 57% of complaints were lodged by advocates and 71% of complaints related to poor communication or to the treatment provided. In 97% of occasions, an explanation and/or an apology resulted. To date, no complaint has proceeded to litigation. Complaints are potentially useful quality assurance tools and can identify remediable system flaws. Health professionals and employers should understand why patients complain and be able to respond appropriately.

[Hemoglobinopathies and patients with foreign names].

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Lilleholt, Kristin; Hallberg, Marit H; Hagve, Tor-Arne

2005-05-04

The diagnosis of haemoglobinopathies is of growing importance in Norway because of increasing immigration from countries where haemoglobinopathies are prevalent conditions. The aim of this study was to investigate the relationship between mean corpuscular volume (MCV) and the various haemoglobinopathies diagnosed in Norway. For a period of three years, all samples with MCV lower than 70 fl were also examined for beta-thalassaemia and haemoglobin variants HbS, HbC, HbE and HbD. A total of 263 samples with low MCV were analysed by high-pressure liquid chromatography. In 18% of the samples, a variant of haemoglobinopathy was found, mainly beta-thalassemia minor. 119 of the samples were from persons with an ethnic background from a country in which these diseases are common; all observed haemoglobinopathies were found in this group. 35% of persons with low MCV and a mainly African or Asian ethnic origin had a heterozygous haeomglobinopathy. Low MCV in patients with a foreign ethnic origin is a useful first step in the diagnosis of haemoglobinopathies.

No association between serum cholesterol and death by suicide in patients with schizophrenia, bipolar affective disorder, or major depressive disorder.

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Park, Subin; Yi, Ki Kyoung; Na, Riji; Lim, Ahyoung; Hong, Jin Pyo

2013-12-05

Previous research on serum total cholesterol and suicidality has yielded conflicting results. Several studies have reported a link between low serum total cholesterol and suicidality, whereas others have failed to replicate these findings, particularly in patients with major affective disorders. These discordant findings may reflect the fact that studies often do not distinguish between patients with bipolar and unipolar depression; moreover, definitions and classification schemes for suicide attempts in the literature vary widely. Subjects were patients with one of the three major psychiatric disorders commonly associated with suicide: schizophrenia, bipolar affective disorder, and major depressive disorder (MDD). We compared serum lipid levels in patients who died by suicide (82 schizophrenia, 23 bipolar affective disorder, and 67 MDD) and non-suicide controls (200 schizophrenia, 49 bipolar affective disorder, and 175 MDD). Serum lipid profiles did not differ between patients who died by suicide and control patients in any diagnostic group. Our results do not support the use of biological indicators such as serum total cholesterol to predict suicide risk among patients with a major psychiatric disorder.

Quantifying the healthcare costs of treating severely bleeding major trauma patients: a national study for England.

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Campbell, Helen E; Stokes, Elizabeth A; Bargo, Danielle N; Curry, Nicola; Lecky, Fiona E; Edwards, Antoinette; Woodford, Maralyn; Seeney, Frances; Eaglestone, Simon; Brohi, Karim; Gray, Alastair M; Stanworth, Simon J

2015-07-06

Severely bleeding trauma patients are a small proportion of the major trauma population but account for 40% of all trauma deaths. Healthcare resource use and costs are likely to be substantial but have not been fully quantified. Knowledge of costs is essential for developing targeted cost reduction strategies, informing health policy, and ensuring the cost-effectiveness of interventions. In collaboration with the Trauma Audit Research Network (TARN) detailed patient-level data on in-hospital resource use, extended care at hospital discharge, and readmissions up to 12 months post-injury were collected on 441 consecutive adult major trauma patients with severe bleeding presenting at 22 hospitals (21 in England and one in Wales). Resource use data were costed using national unit costs and mean costs estimated for the cohort and for clinically relevant subgroups. Using nationally available data on trauma presentations in England, patient-level cost estimates were up-scaled to a national level. The mean (95% confidence interval) total cost of initial hospital inpatient care was £19,770 (£18,177 to £21,364) per patient, of which 62% was attributable to ventilation, intensive care, and ward stays, 16% to surgery, and 12% to blood component transfusion. Nursing home and rehabilitation unit care and re-admissions to hospital increased the cost to £20,591 (£18,924 to £22,257). Costs were significantly higher for more severely injured trauma patients (Injury Severity Score ≥15) and those with blunt injuries. Cost estimates for England were £148,300,000, with over a third of this cost attributable to patients aged 65 years and over. Severely bleeding major trauma patients are a high cost subgroup of all major trauma patients, and the cost burden is projected to rise further as a consequence of an aging population and as evidence continues to emerge on the benefits of early and simultaneous administration of blood products in pre-specified ratios. The findings from

Ferrochelatase deficiency of the bone marrow in a syndrome of congenital microcytic anaemia with iron overload of the liver and hyperferraemia

International Nuclear Information System (INIS)

Stavem, P.; Hovig, T.; Rootwelt, K.; Emblem, R.; Romslo, I.

1985-01-01

By far the most common mechanisms for hypochromic anaemias are either iron deficiency with a limited production of haem or the thalassaemias with a limited production of peptide chains. Some extremely rare congenital hypochromic anaemias have also been reported, in which iron deficiency or thalassaemia is not the cause. One of them is atransferrinaemia. In another rare type of hereditary, congenital hypochromic anaemia, the patients have hyperferraemia with a near fully saturated total iron binding capacity. In spite of heavy haemosiderin deposits in the liver, the bone marrow haemosiderin is reduced. In our studies which where reported in 1983, we found normal transferrin, Hb electrophoresis was normal, and there were no findings indicating thalassaemia minor or lead intoxication. We suggested that the most likely explanation of the condition was a defect in the iron transport mechanism from transferrin into the erythroid cells in the bone marrow, but at that time we had no method for studying this. During the last few years, more reliable methods have become available for assaying ferrochelatase, the enzyme largely responsible for the incorporation of iron into haem. We have therefore repeated our previous studies (with essentially the same results as reported in 1973), and have also assayed ferrochelatase activity of the bone marrow. (author)

Time perception in patients with major depressive disorder during vagus nerve stimulation.

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Biermann, T; Kreil, S; Groemer, T W; Maihöfner, C; Richter-Schmiedinger, T; Kornhuber, J; Sperling, W

2011-07-01

Affective disorders may affect patients' time perception. Several studies have described time as a function of the frontal lobe. The activating eff ects of vagus nerve stimulation on the frontal lobe might also modulate time perception in patients with major depressive disorder (MDD). Time perception was investigated in 30 patients with MDD and in 7 patients with therapy-resistant MDD. In these 7 patients, a VNS system was implanted and time perception was assessed before and during stimulation. A time estimation task in which patients were asked "How many seconds have passed?" tested time perception at 4 defined time points (34 s, 77 s, 192 s and 230 s). The differences between the estimated and actual durations were calculated and used for subsequent analysis. Patients with MDD and healthy controls estimated the set time points relatively accurately. A general linear model revealed a significant main eff ect of group but not of age or sex. The passing of time was perceived as significantly slower in patients undergoing VNS compared to patients with MDD at all time points (T34: t = − 4.2; df = 35; p differences in time perception with regard to age, sex or polarity of depression (uni- or bipolar). VNS is capable of changing the perception of time. This discovery furthers the basic research on circadian rhythms in patients with psychiatric disorders.

Surrogate markers of visceral fat and response to anti-depressive treatment in patients with major depressive disorder

DEFF Research Database (Denmark)

Tønning, Morten; Petersen, Dorthe; Steglich-Petersen, Marie

2017-01-01

Background: Body mass index (BMI) and body weight have been shown to be associated to treatment outcome in patients with major depressive disorder, but this relationship is not clear. Visceral fat might be an underlying mechanism explaining this relationship. Aims: The aim of this study was to pr......Background: Body mass index (BMI) and body weight have been shown to be associated to treatment outcome in patients with major depressive disorder, but this relationship is not clear. Visceral fat might be an underlying mechanism explaining this relationship. Aims: The aim of this study...... was to prospectively investigate whether visceral fat, as measured by hip-to-waist ratio and waist circumference, affects treatment outcome in patients with major depressive disorder in patients attending a hospital psychiatric care unit in Denmark. Methods: The study was conducted as an observational prospective......) interviews and HAM-D6 self-rating questionnaires. Results: No differences were found in outcome between groups of patients with high vs low visceral fat in this population. Conclusions: The lack of association was evident for all surrogate markers of visceral fat, and suggests that visceral fat has no impact...

Mindfulness, Quality of Life, and Severity of Depressive Symptoms Among Patients With Schizophrenia and Patients With Major Depressive Disorder.

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Rayan, Ahmad Hussien Rateb

2017-05-01

The current study used a descriptive correlational design to examine the relationship between mindfulness and quality of life (QOL) among patients with schizophrenia (n = 160) and patients with major depressive disorder (MDD) (n = 161), controlling for demographic and clinical variables. Participants completed self-reported questionnaires regarding demographic variables, severity of depression, QOL, and mindfulness. Patients diagnosed with MDD had higher mindfulness scores than patients diagnosed with schizophrenia. Mindfulness scores were significantly associated with the severity of depression among participants. After controlling for the demographic variables and severity of depressive symptoms, mindfulness had a unique variance in QOL among patients with schizophrenia, but not among patients with MDD. The current study provides preliminary evidence regarding the role of mindfulness in improving depressive symptoms and the overall QOL among patients diagnosed with mental illness. [Journal of Psychosocial Nursing and Mental Health Services, 55(5), 40-50.]. Copyright 2017, SLACK Incorporated.

Study on resting-state fMRI based on amplitude of low-frequency fluctuation in patients with major depression

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Meng-jie PAN

2018-04-01

Full Text Available Objective To observe characteristics of resting-state functional magnetic resonance imaging (rs-fMRI in patients with major depression and explore the possible pathogenesis. Methods A total of 24 major depression patients and 26 sex-, age- and education-matched healthy controls were scanned with rs-fMRI based on amplitude of low-frequency fluctuation (ALFF. The correlation between mALFF values of brain regions and Hamilton Depression Rating Scale-17 (HAMD-17 score was analyzed by Spearman rank correlation analysis. Results Compared with control group, mALFF values in bilateral dorsolateral prefrontal cortex (DLPFC, right orbital superior frontal gyrus, right inferior temporal gyrus, left operculum inferior frontal gyrus, left medial superior frontal gyrus and left gyrus rectus in major depression group were significantly increased (P 0.05, for all. Conclusions Abnormal brain spontaneous activity within default mode network (DMN and limbic system could emerge in major depression patients during resting-state, which may be neurobiological substrate of major depression. DOI: 10.3969/j.issn.1672-6731.2018.03.005

[Long-term effects of combined therapy in patients with beta-thalassemia major].

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Bagnulo, S; Giannini, A M; Moscatelli, F; Stragapede, L; Acquafredda, A; Dammacco, A

1998-01-01

We evaluated therapy complications in 19 beta-thalassemia major patients (mean age from 3 years/5 months and 1 years/6 months) who were followed at II Pediatric Department-University of Bari. 3 out of 19 patients underwent allogenic BMT from matched related donor; 2 out of 19 underwent splenectomy. All of them were receiving hypertransfusion therapy and continuous chelation with DFO. In all patients we performed physical examination, laboratory assays, cardiac and endocrinologic function tests, serum HBV-HCV-HIV antibodies, otoscopy and audiometric test, fundus oculi, skeletal x-ray. 1 out of 19 patients, who was under 15, had a slight dilatation of left ventricle and arythmia. All patients were HBsAb positive. 4/19 patients were HCV Ab positive (ELISA test) with an increase in ALT-AST serum levels since at least 6 months. In 3 of them we assessed RIBA test, always positive. 3 of them underwent liver biopsy (1 iron overload 2 chronic active hepatitis). All patients were HIV Ab negative. 4/15 patients revealed low GH levels after Arginina test. 13 pre-pubescent patients had normal results with GNRH test but lower results after FSH test. 1 pubescent patient had gonadotropic hypophyseal deficit. 4 patients had subclinic hypothiroidism. We couldn't find any sequelas in bone-eyes-ears. Hypertransfusion therapy, chelation, profilaxis of infections improved length and quality of life in thalassemic patients. Hypogonadotropic hypogonadism remains a serious sequela and we think it needs to be treated.

Side effects of Deferasirox Iron Chelation in Patients with Beta Thalassemia Major or Intermedia

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Murtadha Al-Khabori

2013-03-01

Full Text Available Objectives: Chelating agents remain the mainstay in reducing the iron burden and extending patient survival in homozygous beta-thalassemia but adverse and toxic effects may increase with the institution and long term use of this essential therapy. This study aimed to estimate the incidence of deferasirox (DFX side effects in patients with thalassemia major or intermedia.Methods: A retrospective study of 72 patients (mean age: 20.3±0.9 yrs; 36 male, 36 female with thalassemia major or intermedia treated at Sultan Qaboos University Hospital, Oman, was performed to assess the incidence of side effects related to deferasirox over a mean of 16.7 month follow-up period.Results: Six patients experienced rashes and 6 had gastro-intestinal upset. DFX was discontinued in 18 patients for the following reasons: persistent progressive rise(s in serum creatinine (7 patients; 40% mean serum creatinine rise from baseline, feeling unwell (2, severe diarrhea (1, pregnancy (1, death unrelated to chelator (2 and rise in serum transaminases (2. Three patients were reverted to desferoxamine and deferiprone combination therapy as DFX was no longer biochemically effective after 18 months of therapy. There was no correlation between baseline serum ferritin and serum creatinine or a rise in serum creatinine. Cardiac MRI T2* did not change with DFX therapy. However, there was an improvement in liver MRI T2* (p=0.013.Conclusion: Renal side effects related to deferasirox appear to be higher than those reported in published clinical trials. Further larger studies are required to confirm these findings.

Caplacizumab reduces the frequency of major thromboembolic events, exacerbations and death in patients with acquired thrombotic thrombocytopenic purpura.

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Peyvandi, F; Scully, M; Kremer Hovinga, J A; Knöbl, P; Cataland, S; De Beuf, K; Callewaert, F; De Winter, H; Zeldin, R K

2017-07-01

Essentials Acquired thrombotic thrombocytopenic purpura (aTTP) is linked with significant morbidity/mortality. Caplacizumab's effect on major thromboembolic (TE) events, exacerbations and death was studied. Fewer caplacizumab-treated patients had a major TE event, an exacerbation, or died versus placebo. Caplacizumab has the potential to reduce the acute morbidity and mortality associated with aTTP. Background Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk for thrombotic complications, exacerbations, and death. In the phase II TITAN study, treatment with caplacizumab, an anti-von Willebrand factor Nanobody ® was shown to reduce the time to confirmed platelet count normalization and exacerbations during treatment. Objective The clinical benefit of caplacizumab was further investigated in a post hoc analysis of the incidence of major thromboembolic events and exacerbations during the study drug treatment period and thrombotic thrombocytopenic purpura-related death during the study. Methods The Standardized Medical Dictionary for Regulatory Activities (MedDRA) Query (SMQ) for 'embolic and thrombotic events' was run to investigate the occurrence of major thromboembolic events and exacerbations in the safety population of the TITAN study, which consisted of 72 patients, of whom 35 received caplacizumab and 37 received placebo. Results Four events (one pulmonary embolism and three aTTP exacerbations) were reported in four patients in the caplacizumab group, and 20 such events were reported in 14 patients in the placebo group (two acute myocardial infarctions, one ischemic stroke, one hemorrhagic stroke, one pulmonary embolism, one deep vein thrombosis, one venous thrombosis, and 13 aTTP exacerbations). Two of the placebo-treated patients died from aTTP during the study. Conclusion In total, 11.4% of caplacizumab

The quality of life of hematological malignancy patients with major depressive disorder or subsyndromal depression.

Science.gov (United States)

Rezaei, Omid; Sharifian, Ramezan-Ali; Soleimani, Mehdi; Jahanian, Amirabbas

2012-01-01

The purpose of the present study was to compare the quality of life of hematological malignancy patients with major depressive disorder or subsyndromal depression. Sample consisted of 93 hematological malignancy patients recruited from oncology ward of Valieasr hospital for Imam Khomeini complex hospital at Tehran through purposeful sampling. Participants were divided into three groups through diagnostic interview based on DSM-IV-TR criteria and the Beck Depression Inventory-2 (BDI-II): Major depressive disorder (MDD) (n = 41; 44.1%); subsyndromal depression (SSD) (n = 23; 24.7%), and without depression (WD) (n = 29; 31.2%). Participants completed the short-form health survey (SF-36) as a measure of the quality of life. We carried out an analysis of covariance to examine the collected data. Findings showed that there was not a significant difference between patients with MDD and SSD based on measure of quality of life. But patients with MDD and SSD showed significantly worse quality of life than patients with WD. This finding highlights the clinical importance of subsyndromal depressive symptoms and casts doubt on the clinical utility of separation between MDD and subsyndromal depression in terms of important clinical outcomes.

Risk of subsequent dementia among patients with bipolar disorder or major depression: a nationwide longitudinal study in Taiwan.

Science.gov (United States)

Chen, Mu-Hong; Li, Cheng-Ta; Tsai, Chia-Fen; Lin, Wei-Chen; Chang, Wen-Han; Chen, Tzeng-Ji; Pan, Tai-Long; Su, Tung-Ping; Bai, Ya-Mei

2015-06-01

Both major depression and bipolar disorder are associated with an increased risk of developing dementia. However, the differential risk of dementia between major depression and bipolar disorder is rarely investigated. Using the Taiwan National Health Insurance Research Database, a total of 2291 patients aged ≥ 55 years (major depression: 1946 and bipolar disorder: 345) and 2291 age-and sex-matched controls were enrolled between 1998 and 2008, and followed to the end of 2011. Participants who developed dementia during the follow-up were identified. Both patients with bipolar disorder [hazard ratio (HR) 5.58, 95% confidence interval (CI) 4.26-7.32] and those with major depression (HR 3.02, 95% CI 2.46-3.70) had an increased risk of developing dementia in later life, after adjusting for demographic data and medical comorbidities. The sensitivity tests after excluding the 1-year (bipolar disorder: HR 4.73, 95% CI 3.50-6.35; major depression: HR 2.62, 95% CI 2.11-3.25) and 3-year (HR 3.92, 95% CI 2.78-5.54; HR 2.21, 95% CI 1.73-2.83, respectively) follow-up duration also revealed consistent findings. Furthermore, patients with bipolar disorder were associated with an 87% increased risk (HR 1.87, 95% CI 1.48-2.37) of subsequent dementia compared with patients with major depression. Midlife individuals with bipolar disorder or major depression were associated with an elevated risk of developing dementia in later life. Further studies may be required to clarify the underlying mechanisms among major depression, bipolar disorder, and dementia, and to investigate whether prompt intervention may decrease this risk. Copyright © 2015 AMDA – The Society for Post-Acute and Long-Term Care Medicine. Published by Elsevier Inc. All rights reserved.

Sustained major molecular response on interferon alpha-2b in two patients with polycythemia vera

DEFF Research Database (Denmark)

Larsen, T.S.; Pallisgaard, N.; Andersen, M.T.

2008-01-01

with a JAK2 V617F allele burden below 1.0% in two patients with polycythemia vera treated with interferon alpha-2b (IFN-2b). Discontinuation of IFN-2b in one of the patients was followed by a sustained long-lasting (12 months of follow-up) major molecular response Udgivelsesdato: 2008/10...

Exercise for patients with major depression

DEFF Research Database (Denmark)

Krogh, Jesper; Speyer, Helene; Gluud, Christian

2015-01-01

is to investigate the beneficial and harmful effects of exercise, in terms of severity of depression, lack of remission, suicide, and so on, compared with treatment as usual with or without co-interventions in randomized clinical trials involving adults with a clinical diagnosis of major depression. A meta......BACKGROUND: The lifetime prevalence of major depression is estimated to affect 17% of the population and is considered the second largest health-care problem globally in terms of the number of years lived with disability. The effects of most antidepressant treatments are poor; therefore, exercise...... has been assessed in a number of randomized clinical trials. A number of reviews have previously analyzed these trials; however, none of these reviews have addresses the effect of exercise for adults diagnosed with major depression. METHODS/DESIGN: The objective of this systematic review...

Clinical validity of a population database definition of remission in patients with major depression.

Science.gov (United States)

Sicras-Mainar, Antoni; Blanca-Tamayo, Milagrosa; Gutiérrez-Nicuesa, Laura; Salvatella-Pasant, Jordi; Navarro-Artieda, Ruth

2010-02-11

Major depression (MD) is one of the most frequent diagnoses in Primary Care. It is a disabling illness that increases the use of health resources. To describe the concordance between remission according to clinical assessment and remission obtained from the computerized prescription databases of patients with MD in a Spanish population. multicenter cross-sectional. The population under study was comprised of people from six primary care facilities, who had a MD episode between January 2003 and March 2007. A specialist in psychiatry assessed a random sample of patient histories and determined whether a certain patient was in remission according to clinical criteria (ICPC-2). Regarding the databases, patients were considered in remission when they did not need further prescriptions of AD for at least 6 months after completing treatment for a new episode. Validity indicators (sensitivity [S], specificity [Sp]) and clinical utility (positive and negative probability ratio [PPR] and [NPR]) were calculated. The concordance index was established using Cohen's kappa coefficient. Significance level was p Reliability analysis: Cronbach's alpha: 90.6% (CI was 95%: 85.6 - 95.6%). Results show an acceptable level of concordance between remission obtained from the computerized databases and clinical criteria. The major discrepancies were found in diagnostic accuracy.

Risk of major depression in patients with chronic renal failure on different treatment modalities: A matched-cohort and population-based study in Taiwan.

Science.gov (United States)

Chen, Shih-Feng; Wang, I-Jen; Lang, Hui-Chu

2016-01-01

The influence of different treatment modalities on the risk of developing major depression in patients with chronic renal failure (CRF) is not well understood. We aimed to explore the incidence of major depression among patients with CRF who were on different dialysis modalities, who had received renal transplantation (RT), and those who had not yet received any of the aforementioned renal replacement therapies. We conducted a population-based retrospective cohort study using a national health insurance research database. This study investigated 89,336 study controls, 17,889 patients with chronic kidney disease on conservative treatment, 3823 patients on hemodialysis (HD), 351 patients on peritoneal dialysis (PD), and 322 patients who had RT. We followed all individuals until the occurrence of major depression or the date of loss to follow-up. The PD group had the highest risk (hazard ratio [HR] 2.43; 95% confidence interval [CI] 1.26-4.69), whereas the RT group had the lowest risk (HR 0.18; 95% CI 0.03-1.29) of developing major depression compared with the control group. Patients initiated on PD had a higher risk of developing major depression than patients initiated on HD (pairwise comparison: HR 2.20; 95% CI 1.09-4.46). Different treatment modalities are associated with different risks of developing major depression in patients with CRF. Among renal replacement therapies, patients who have had RT have the lowest risk of developing major depression. Patients who initiate renal therapy on PD may have a higher risk of major depression compared with patients who initiate renal therapy on HD. © 2015 International Society for Hemodialysis.

The Impact of Two Different Transfusion Strategies on Patient Immune Response during Major Abdominal Surgery: A Preliminary Report

OpenAIRE

Theodoraki, Kassiani; Markatou, Maria; Rizos, Demetrios; Fassoulaki, Argyro

2014-01-01

Blood transfusion is associated with well-known risks. We investigated the difference between a restrictive versus a liberal transfusion strategy on the immune response, as expressed by the production of inflammatory mediators, in patients subjected to major abdominal surgery procedures. Fifty-eight patients undergoing major abdominal surgery were randomized preoperatively to either a restrictive transfusion protocol or a liberal transfusion protocol (with transfusion if hemoglobin dropped be...

Multi-centre diagnostic classification of individual structural neuroimaging scans from patients with major depressive disorder.

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Mwangi, Benson; Ebmeier, Klaus P; Matthews, Keith; Steele, J Douglas

2012-05-01

Quantitative abnormalities of brain structure in patients with major depressive disorder have been reported at a group level for decades. However, these structural differences appear subtle in comparison with conventional radiologically defined abnormalities, with considerable inter-subject variability. Consequently, it has not been possible to readily identify scans from patients with major depressive disorder at an individual level. Recently, machine learning techniques such as relevance vector machines and support vector machines have been applied to predictive classification of individual scans with variable success. Here we describe a novel hybrid method, which combines machine learning with feature selection and characterization, with the latter aimed at maximizing the accuracy of machine learning prediction. The method was tested using a multi-centre dataset of T(1)-weighted 'structural' scans. A total of 62 patients with major depressive disorder and matched controls were recruited from referred secondary care clinical populations in Aberdeen and Edinburgh, UK. The generalization ability and predictive accuracy of the classifiers was tested using data left out of the training process. High prediction accuracy was achieved (~90%). While feature selection was important for maximizing high predictive accuracy with machine learning, feature characterization contributed only a modest improvement to relevance vector machine-based prediction (~5%). Notably, while the only information provided for training the classifiers was T(1)-weighted scans plus a categorical label (major depressive disorder versus controls), both relevance vector machine and support vector machine 'weighting factors' (used for making predictions) correlated strongly with subjective ratings of illness severity. These results indicate that machine learning techniques have the potential to inform clinical practice and research, as they can make accurate predictions about brain scan data from

rCBF change in the brain of patients with major depressive disorder

International Nuclear Information System (INIS)

Sun Da; Xu Wei; Zhan Hongwei; Liu Hongbiao

2010-01-01

Purpose Major depressive disorder is a frequent emotional mood disorder. To evaluate the changes of brain blood flow in patients with depressive disorder and the correlation between rCBF and clinical feature is very important to diagnosis and treatment of this decease. Methods: Regional cerebral perfusion was investigated using SPECT in 75 patients with depressive disorders. The mean ages of the patients were 41.9 (17-74) Years old. The course of disease was different from several days to over 20 years. Results: 97.3 per cent of patients (73/75) had relative hypoperfusions in some cerebral regions. The patients had a significant decrease of rCBF in the frontal lobesbilaterally, and temporal lobes, basal ganglia, thalamus and parietal lobe. The course of disease and age of the patients had a negative correlation with the changes of rCBF. Conclusion: According to the results of our study, patients with depressive disorders had profound dysfunction of the frontal lobes bilaterally. The temporal cortices and basal ganglia were involved in most patients too. It is coincident with the results of other studies. The function of frontal lobes and temporal lobes is close relation close with affective action, attention, memory, thinking, abstraction, and other brain cognitive function. The clinical symptom of depressive disorder may be relevant with hypoperfusions of frontal lobes and temporal lobes. (authors)

Age-related postoperative morphine requirements in children following major surgery--an assessment using patient-controlled analgesia (PCA)

DEFF Research Database (Denmark)

Hansen, Tom Giedsing; Henneberg, Steen Winther; Hole, P

1996-01-01

To investigate if small children require less morphine for postoperative analgesia than do older children and adolescents we analysed the morphine consumption pattern of 28 consecutive children on intravenous patient-controlled analgesia (PCA) following major surgery. The median age-specific morp......To investigate if small children require less morphine for postoperative analgesia than do older children and adolescents we analysed the morphine consumption pattern of 28 consecutive children on intravenous patient-controlled analgesia (PCA) following major surgery. The median age...

Functional impairment in patients with major depressive disorder: the 2-year PERFORM study.

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Hammer-Helmich, Lene; Haro, Josep Maria; Jönsson, Bengt; Tanguy Melac, Audrey; Di Nicola, Sylvie; Chollet, Julien; Milea, Dominique; Rive, Benoît; Saragoussi, Delphine

2018-01-01

The Prospective Epidemiological Research on Functioning Outcomes Related to Major depressive disorder (PERFORM) study describes the course of depressive symptoms, perceived cognitive symptoms, and functional impairment over 2 years in outpatients with major depressive disorder (MDD) and investigates the patient-related factors associated with functional impairment. This was a 2-year observational study in 1,159 outpatients with MDD aged 18-65 years who were either initiating antidepressant monotherapy or undergoing their first switch of antidepressant. Functional impairment was assessed by the Sheehan Disability Scale and the Work Productivity and Activity Impairment questionnaire. Patients assessed depression severity using the nine-item Patient Health Questionnaire and severity of perceived cognitive symptoms using the five-item Perceived Deficit Questionnaire. To investigate which patient-related factors were associated with functional impairment, univariate analyses of variance were performed to identify relevant factors that were then included in multivariate analyses of covariance at baseline, month 2, months 6 and 12 combined, and months 18 and 24 combined. The greatest improvement in depressive symptoms, perceived cognitive symptoms, and functional impairment was seen immediately (within 2 months) following initiation or switch of antidepressant therapy, followed by more gradual improvement and long-term stabilization. Improvement in perceived cognitive symptoms was less marked than improvement in depressive symptoms during the acute treatment phase. Functional impairment in patients with MDD was not only associated with severity of depressive symptoms but also independently associated with severity of perceived cognitive symptoms when adjusted for depression severity throughout the 2 years of follow-up. These findings highlight the burden of functional impairment in MDD and the importance of recognizing and managing cognitive symptoms in daily practice.

Nutritional Status in Patients with Major Depressive Disorders: A Pilot Study in Tabriz, Iran

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Bahram Pourghassem Gargari

2012-12-01

Full Text Available Introduction: This study was conducted to assess the nutritional status in Iranian major depres-sive disorder patients. We also determined the relationship between nutrients intake with depres-sion severity.Methods: Seventy major depressive patients were selected randomly from outpatient depressive subjects, referred to Razi Psychiatry Hospital in Tabriz, Iran in 2007. Dietary intakes were rec-orded and compared with dietary reference intakes (DRIs. Definition of the disease and its se-verity were according to DSM-IV-TR and Hamilton Depression Rating Scale, respectively. Nu-tritionist III program, Chi-square, correlation and t-test were used for data analyses. Demo-graphic, clinical and laboratory data were analyzed using SPSS software for windows (ver-sion13.0.Results: According to dietary analysis, 11.4% and 55% of patients had dietary protein and energy deficiency, respectively. 97.1% and 95.7% of patients had less folate and B12 intakes than recom-mended dietary allowances. The mean (Mean ± SD for plasma folate and B12 was 5.18±6.11 ng/ml and 389.05±346.9 pg/ml, respectively. Low plasma folate and B12 was observed in 51.4% and 50.0 % of patients, respectively. There was no significant relationship between blood folate and B12 levels with depression severity. Similarly, nutrients intake had no effect on depression se-verity.Conclusions: Low plasma concentrations and low dietary intakes of folate and B12 are common among Tabrizian depressive patients. It seems that nutritional intervention for increasing folate and vitamin B12 intake must be considered as health promotive and preventative program for pa-tients suffering from depression disorders.

Clinical validity of a population database definition of remission in patients with major depression

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Salvatella-Pasant Jordi

2010-02-01

Full Text Available Abstract Background Major depression (MD is one of the most frequent diagnoses in Primary Care. It is a disabling illness that increases the use of health resources. Aim: To describe the concordance between remission according to clinical assessment and remission obtained from the computerized prescription databases of patients with MD in a Spanish population. Methods Design: multicenter cross-sectional. The population under study was comprised of people from six primary care facilities, who had a MD episode between January 2003 and March 2007. A specialist in psychiatry assessed a random sample of patient histories and determined whether a certain patient was in remission according to clinical criteria (ICPC-2. Regarding the databases, patients were considered in remission when they did not need further prescriptions of AD for at least 6 months after completing treatment for a new episode. Validity indicators (sensitivity [S], specificity [Sp] and clinical utility (positive and negative probability ratio [PPR] and [NPR] were calculated. The concordance index was established using Cohen's kappa coefficient. Significance level was p Results 133 patient histories were reviewed. The kappa coefficient was 82.8% (confidence intervals [CI] were 95%: 73.1 - 92.6, PPR 9.8% and NPR 0.1%. Allocation discrepancies between both criteria were found in 11 patients. S was 92.5% (CI was 95%: 88.0 - 96.9% and Sp was 90.6% (CI was 95%: 85.6 - 95.6%, p Conclusions Results show an acceptable level of concordance between remission obtained from the computerized databases and clinical criteria. The major discrepancies were found in diagnostic accuracy.

Thalassaemic osteopathy: a cross-sectional preliminary study from Sri Lanka.

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Dissanayake, Ruwangi; de Silva, Shamya; Lekamwasam, Sarath; Abeysekara, Gayan; Dissanayake, Vajira H W

2014-03-01

The objectives of this study were to demonstrate the presence of low bone mineral density (BMD) and to examine the association of known risk factors for low BMD in patients with beta thalassaemia major in Sri Lanka. Thirty-eight patients were studied. Their examination and laboratory investigation findings were recorded (haematology, biochemistry, hormonal profile, COL1A1 rs1800012G>T genotype, dual energy X-ray absorptiometry (DXA) scanning). Mean age was 10.95 years (range 5-21.4). 20 (52.6%) were male. BMD was low (z score ≤-2.0) in 12 (31.5%). Regression analysis of BMD on known risk factors (age, sex, pubertal stage, ferritin level, average pre-transfusion haemoglobin, serum calcium level and COL1A1 rs1800012G>T genotype) controlling for confounding factors on each comparison, showed that only age was significantly associated with low BMD (p=0.005). Approximately one-third of patients had a low BMD. Only age was significantly associated with a low BMD.

Long-term outcome of major depressive disorder in psychiatric patients is variable.

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Holma, K Mikael; Holma, Irina A K; Melartin, Tarja K; Rytsälä, Heikki J; Isometsä, Erkki T

2008-02-01

The prevailing view of outcome of major depressive disorder (MDD), based on mostly inpatient cohorts sampled from tertiary centers, emphasizes chronicity and frequent recurrences. We investigated the long-term outcome of a regionally representative psychiatric MDD cohort comprising mainly outpatients. The Vantaa Depression Study included 163 patients with DSM-IV MDD (71.5% of those eligible) diagnosed using structured and semistructured interviews and followed up at 6 months, 18 months, and 5 years with a life chart between February 1, 1997, and April 30, 2004. The effects of comorbid disorders and other predictors on outcome were comprehensively investigated. Over the 5-year follow-up, 98.8% of patients achieved a symptom state below major depressive episode (MDE) criteria, and 88.4% reached full remission, with the median time to full remission being 11.0 months. Nearly one third (29.3%) had no recurrences, whereas 30.0% experienced 1, 12.9% experienced 2, and 27.9% experienced 3 or more recurrences. Preceding dysthymic disorder (p = .028), cluster C personality disorder (p = .041), and longer MDE duration prior to entry (p = .011) were the most significant predictors of longer time in achieving full remission. Severity of MDD and comorbidity, especially social phobia, predicted probability of, shorter time to, and number of recurrences. Previous literature on mostly inpatient MDD may have, by generalizing from patients with the most severe psychopathology, overemphasized chronicity of MDD. The long-term outcome of MDD in psychiatric care is variable, with about one tenth of patients having poor, one third having intermediate, and one half having favorable outcomes. In addition to known predictors, cluster C personality disorders and social phobia warrant further attention as predictors of MDD outcome among outpatients.

A pilot study on predictors of brainstem raphe abnormality in patients with major depressive disorder.

Science.gov (United States)

Kostić, Milutin; Munjiza, Ana; Pesic, Danilo; Peljto, Amir; Novakovic, Ivana; Dobricic, Valerija; Tosevski, Dusica Lecic; Mijajlovic, Milija

2017-02-01

Hypo/anechogenicity of the brainstem raphe (BR) structures has been suggested as a possible transcranial parenchymal sonography (TCS) marker associated with depression. The aim of this study was to analyze possible association of the abnormal BR echogenicity in patients with major depression when compared to healthy controls, and to evaluate its clinical and genetic correlates. TCS was performed in 53 patients diagnosed as major depressive disorder (MDD) without psychotic symptoms and in 54 healthy matched controls. The TCS detected BR abnormalities were significantly more frequent in MDD patients (35 out of 53; 66%) in comparison to matched controls (5 out of 56; 9%). The prevalence of short allele (s) homozygocity in the length polymorphism of the promoter region of the serotonin transporter gene (5-HTTLPR) was significantly higher in MDD patients relative to those with normal BR echogenicity. A stepwise statistical discriminant analysis revealed statistically significant separation between MDD patients with and without BR abnormalities groups based on the four predictors combined: the Hamilton Anxiety Rating Scale item 5 ("difficulty in concentration, poor memory"), presence of social phobia, s allele homozygocity of the 5-HTTLPR polymorphism, and presence of generalized anxiety disorder. Cross-sectional design and heterogenous treatment of depressed patients. Reduced BR echogenicity in at least a subgroup of MDD patients may reflect a particular phenotype, characterized by more prevalent comorbid anxiety disorders, associated with particular genetic polymorphisms and neurotransmitter(s) deficits, most probably altered serotonergic mechanisms. Copyright © 2016 Elsevier B.V. All rights reserved.

Consanguinity Ratio in Beta-Thalassemia Major Patients in District Bannu

International Nuclear Information System (INIS)

Khan, M. S.; Ahmed, M.; Khan, R. A.; Mushtaq, N.; Shah, M. W. U.

2015-01-01

Objective: To assess the frequency of consanguinity in b-thalassemia major patients and its association with age, gender and hepatitis C virus antibody positivity. Methods: The cross-sectional study was conducted from June 2013 to July 2014 at various hospitals of district Bannu in the North Western Khyber Pakhtunkhwa province of Pakistan. Data was recorded on a predesigned questionnaire. Results: Out of 180 subjects, 133(74 percent) parents were cousins, while 47(26 percent) were unrelated. The frequency of anti-hepatitis C virus antibody positivity was 14(7.77 percent). Conclusion: High prevalence of the disease in the study region was due to consanguineous marriages. (author)

Kidney bean: a major sensitizer among legumes in asthma and rhinitis patients from India.

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Ramkrashan Kasera

Full Text Available BACKGROUND: The prevalence of IgE mediated food allergies has increased over the last two decades. Food allergy has been reported to be fatal in highly sensitive individuals. Legumes are important food allergens but their prevalence may vary among different populations. The present study identifies sensitization to common legumes among Indian population, characterizes allergens of kidney bean and establishes its cross reactivity with other legumes. METHODOLOGY: Patients (n = 355 with history of legume allergy were skin prick tested (SPT with 10 legumes. Specific IgE (sIgE and total IgE were estimated in sera by enzyme-linked immunosorbent assay. Characterization of kidney bean allergens and their cross reactivity was investigated by immunobiochemical methods. Identification of major allergens of kidney bean was carried out by mass spectrometry. PRINCIPAL FINDINGS: Kidney bean exhibited sensitization in 78 (22.0% patients followed by chickpea 65 (18.0% and peanut 53 (15%. SPT positive patients depicted significantly elevated sIgE levels against different legumes (r = 0.85, p<0.0001. Sera from 30 kidney bean sensitive individuals exhibited basophil histamine release (16-54% which significantly correlated with their SPT (r = 0.83, p<0.0001 and sIgE (r = 0.99, p<0.0001. Kidney bean showed eight major allergens of 58, 50, 45, 42, 40, 37, 34 and 18 kDa on immunoblot and required 67.3±2.51 ng of homologous protein for 50% IgE inhibition. Inhibition assays revealed extensive cross reactivity among kidney bean, peanut, black gram and pigeon pea. nLC-MS/MS analysis identified four allergens of kidney bean showing significant matches with known proteins namely lectin (phytohemagglutinin, phaseolin, alpha-amylase inhibitor precursor and group 3 late embryogenesis abundant protein. CONCLUSION/SIGNIFICANCE: Among legumes, kidney bean followed by chick pea and peanut are the major allergic triggers in asthma and rhinitis patients in India

Survival times of patients with a first hip fracture with and without subsequent major long-bone fractures.

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Angthong, Chayanin; Angthong, Wirana; Harnroongroj, Thos; Naito, Masatoshi; Harnroongroj, Thossart

2013-01-01

Survival rates are poorer after a second hip fracture than after a first hip fracture. Previous survival studies have included in-hospital mortality. Excluding in-hospital deaths from the analysis allows survival times to be evaluated in community-based patients. There is still a lack of data regarding the effects of subsequent fractures on survival times after hospital discharge following an initial hip fracture. This study compared the survival times of community-dwelling patients with hip fracture who had or did not have a subsequent major long-bone fracture. Hazard ratios and risk factors for subsequent fractures and mortality rates with and without subsequent fractures were calculated. Of 844 patients with hip fracture from 2000 through 2008, 71 had a subsequent major long-bone fracture and 773 did not. Patients who died of other causes, such as perioperative complications, during hospitalization were excluded. Such exclusion allowed us to determine the effect of subsequent fracture on the survival of community-dwelling individuals after hospital discharge or after the time of the fracture if they did not need hospitalization. Demographic data, causes of death, and mortality rates were recorded. Differences in mortality rates between the patient groups and hazard ratios were calculated. Mortality rates during the first year and from 1 to 5 years after the most recent fracture were 5.6% and 1.4%, respectively, in patients with subsequent fractures, and 4.7% and 1.4%, respectively, in patients without subsequent fractures. These rates did not differ significantly between the groups. Cox regression analysis and calculation of hazard ratios did not show significant differences between patients with subsequent fractures and those without. On univariate and multivariate analyses, age fracture. This study found that survival times did not differ significantly between patients with and without subsequent major long-bone fractures after hip fracture. Therefore, all

Relationship between personality and disability in patients with major depressive disorder.

Science.gov (United States)

Güleç, Medine Yazici; Hocaoğlu, Ciçek

2011-01-01

The co-morbidity of major depressive disorder (MDD ) with personality disorders (PDs) in patients with long-standing work disability at a psychiatry clinic was investigated. The purpose of our study was to evaluate personality for contributing to disability in patients with MDD and to investigate the relationship with these two psychometric characters in patients with MDD. Seventy-two patients with a MDD and 72 healthy controls were assessed by means of both clinician and self-rating scales for depression, anxiety, disability, and the SCID-II personality inventory. There was no difference between the personality parameters of the groups regarding schizotypal and antisocial PDs. Avoidant personality was found to be less common in the patient group (p=0.030). Dependent (p less than 0.001), obsessive (p=0.003), passive-aggressive (p=0.025), self-defeating (p less than 0.001), paranoid (p less than 0.001), schizoid (p=0.012), histrionic (p=0.001), narcissistic (p less than 0.001), and borderline (p less than 0.001) PDs in patients were more common than in controls. On the disability sub-scales, physical role limitation, vitality, social functioning, emotional role limitation, and mental health were significantly lower in patient group than normal control group. While Cluster A was not related to any disability subscale, Cluster B had a positive correlation with vitality and mental health, whereas Cluster C and Cluster NOS had a negative correlation with emotional role limitation. Only the emotional role limitation predicts the presence of depression, whereas only self-defeating, obsessive, paranoid, and passive aggressive personality predict the emotional role limitation. Patients with MDD have personality and disability problems. PDs in depression contribute to disability. Our results demonstrated that the emotional role limitation is the unique sub-scale that predicts the MDD group.

Addressing adherence to treatment: a longstanding concern. The patients’ perspective

OpenAIRE

Mahmoud Hadipour Dehshal

2014-01-01

Adherence to treatment is a great concern for patients who need long-life treatment. Thalassaemia is an inherited disease for whose treatment team-working is of a considerable importance. To logically face the problem of poor compliance patients, all members of the team ought to be aware of the causing factors and the ways to handle the problem. The factors which cause the lack of compliance among patients could be stratified into economic and structural facto. Furthermore, patient-related fa...

Afican Health Sciences Vol 9 No 1.pmd

African Journals Online (AJOL)

Administrator

South-east Asia but they occur sporadically in other populations. The a0 thalassaemias are found most commonly in the Mediterranean and Orientals but are extremely rare in Africa and the Middle East. The deletional forms of a+ thalassaemia however occur with great frequency throughout West Africa, the. Mediterranean ...

Major Odorants Released as Urinary Volatiles by Urinary Incontinent Patients

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In Young Sa

2013-07-01

Full Text Available In this study, volatile urinary components were collected using three different types of samples from patients suffering from urinary incontinence (UI: (1 urine (A; (2 urine + non-used pad (B; and (3 urine + used pad (C. In addition, urine + non-used pad (D samples from non-patients were also collected as a reference. The collection of urinary volatiles was conducted with the aid of a glass impinger-based mini-chamber method. Each of the four sample types (A through D was placed in a glass impinger and incubated for 4 hours at 37 °C. Ultra pure air was then passed through the chamber, and volatile urine gas components were collected into Tedlar bags at the other end. These bag samples were then analyzed for a wide range of VOCs and major offensive odorants (e.g., reduced sulfur compounds (RSCs, carbonyls, trimethylamine (TMA, ammonia, etc.. Among the various odorants, sulfur compounds (methanethiol and hydrogen sulfide and aldehydes (acetaldehyde, butylaldehyde, and isovaleraldehyde were detected above odor threshold and predicted to contribute most effectively to odor intensity of urine incontinence.

C‑reactive Protein and Disease Outcome in Nigerian Sickle Cell ...

African Journals Online (AJOL)

Sillesen H, Nordestgaard BG. Genetically elevated C‑reactive protein and ischemic vascular disease. N Engl J Med. 2008;359:1897‑908. 18. Walter PB, Fung EB, Killilea DW, Jiang Q, Hudes M, Madden J, et al. Oxidative stress and inflammation in iron‑overloaded patients with beta‑thalassaemia or sickle cell disease. Br J.

Prevalence of major depressive disorder in patients receiving beta-blocker therapy versus other medications.

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Carney, R M; Rich, M W; teVelde, A; Saini, J; Clark, K; Freedland, K E

1987-08-01

Depression is believed to be a common side effect in patients receiving beta-blocker therapy. However, diagnoses of depression defined by current diagnostic criteria may not be more common in patients receiving beta-blockers than in patients with the same medical disorder receiving other medications. Seventy-seven patients undergoing elective cardiac catheterization for evaluation of chest pain received a semi-structured diagnostic psychiatric interview. Twenty-one percent of the patients receiving beta-blockers and 33 percent of the patients receiving medications other than beta-blockers met the current American Psychiatric Association criteria for major depressive disorder (DSM-III) (p = NS). The mean heart rate and state anxiety scores for patients taking beta-blockers were significantly lower than those measured in patients taking medications other than beta-blockers. No other medical or demographic differences were observed between the two groups. Despite the methodologic limitations of the study, there does not appear to be a difference in the point prevalence of depression between patients receiving beta-blockers and those receiving other medications.

Does prehospital time affect survival of major trauma patients where there is no prehospital care?

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S B Dharap

2017-01-01

Full Text Available Background: Survival after major trauma is considered to be time dependent. Efficient prehospital care with rapid transport is the norm in developed countries, which is not available in many lower middle and low-income countries. The aim of this study was to assess the effect of prehospital time and primary treatment given on survival of major trauma patients in a setting without prehospital care. Materials and Methods: This prospective observational study was carried out in a university hospital in Mumbai, from January to December 2014. The hospital has a trauma service but no organized prehospital care or defined interhospital transfer protocols. All patients with life- and/or limb-threatening injuries were included in the study. Injury time and arrival time were noted and the interval was defined as “prehospital time” for the directly arriving patients and as “time to tertiary care” for those transferred. Primary outcome measure was in-hospital death (or discharge. Results: Of 1181 patients, 352 were admitted directly from the trauma scene and 829 were transferred from other hospitals. In-hospital mortality was associated with age, mechanism and mode of injury, shock, Glasgow Coma Score <9, Injury Severity Score ≥16, need for intubation, and ventilatory support on arrival; but neither with prehospital time nor with time to tertiary care. Transferred patients had a significantly higher mortality (odds ratio = 1.869, 95% confidence interval = 1.233–2.561, P = 0.005 despite fewer patients with severe injury. Two hundred and ninety-four (35% of these needed airway intervention while 108 (13% needed chest tube insertion on arrival to the trauma unit suggesting inadequate care at primary facility. Conclusion: Mortality is not associated with prehospital time but with transfers from primary care; probably due to deficient care. To improve survival after major trauma, enhancement of resources for resuscitation and capacity building of on

Costs of major intracranial, gastrointestinal and other bleeding events in patients with atrial fibrillation - a nationwide cohort study.

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Jakobsen, Marie; Kolodziejczyk, Christophe; Klausen Fredslund, Eskild; Poulsen, Peter Bo; Dybro, Lars; Paaske Johnsen, Søren

2017-06-12

Use of oral anticoagulation therapy in patients with atrial fibrillation (AF) involves a trade-off between a reduced risk of ischemic stroke and an increased risk of bleeding events. Different anticoagulation therapies have different safety profiles and data on the societal costs of both ischemic stroke and bleeding events are necessary for assessing the cost-effectiveness and budgetary impact of different treatment options. To our knowledge, no previous studies have estimated the societal costs of bleeding events in patients with AF. The objective of this study was to estimate the 3-years societal costs of first-incident intracranial, gastrointestinal and other major bleeding events in Danish patients with AF. The study was an incidence-based cost-of-illness study carried out from a societal perspective and based on data from national Danish registries covering the period 2002-2012. Costs were estimated using a propensity score matching and multivariable regression analysis (first difference OLS) in a cohort design. Average 3-years societal costs attributable to intracranial, gastrointestinal and other major bleeding events were 27,627, 17,868, and 12,384 EUR per patient, respectively (2015 prices). Existing evidence shows that the corresponding costs of ischemic stroke were 24,084 EUR per patient (2012 prices). The average costs of bleeding events did not differ between patients with AF who were on oral anticoagulation therapy prior to the event and patients who were not. The societal costs attributable to major bleeding events in patients with AF are significant. Intracranial haemorrhages are most costly to society with average costs of similar magnitude as the costs of ischemic stroke. The average costs of gastrointestinal and other major bleeding events are lower than the costs of intracranial haemorrhages, but still substantial. Knowledge about the relative size of the costs of bleeding events compared to ischemic stroke in patients with AF constitutes

The majority of Danish nontoxic goitre patients are ineligible for Levothyroxine suppressive therapy

DEFF Research Database (Denmark)

Fast, Søren; Bonnema, Steen Joop; Hegedüs, Laszlo

2008-01-01

evaluated clinically including fine needle aspiration biopsy, thyroid scintigraphy and ultrasound. Seven-hundred and forty-five patients (627 women and 118 men; median age 47 years, range 11-90) were potential candidates for LT4-therapy. Based on guidelines we defined conditions where LT4-therapy......OBJECTIVE: Levothyroxine suppressive therapy (LT4-therapy), aimed at shrinking thyroid nodules is controversial. Despite evidence of limited effect and long-term side-effects, questionnaire surveys indicate widespread use. Our aim was to determine, in consecutive nontoxic goitre patients...... serum TSH, post-menopausal status, a large goitre or clinical suspicion of malignancy. CONCLUSION: The vast majority of consecutive Danish nontoxic goitre patients (84%) were ineligible for LT4-therapy. Due to low efficacy and potential long-term adverse effects on the skeleton and cardiovascular system...

Evaluation of pulmonary function in beta-thalassemia major patients

International Nuclear Information System (INIS)

Eidani, I.; Keikhaei, B.; Rahim, F.; Bagheri, A.

2010-01-01

Objective: To describe and quantify the functional change of the lung in patients with beta-thalassemia major (TM) and determine the correlation between pulmonary function test (PFT) results with hemoglobin, ferritin and age changes. Methodology: Pulmonary function tests were performed on 60 transfusion-dependent patients with TM, ranging in age from 10 to 45 years. Percent-predicted values for forced expiratory volume in one second (FEV1), and forced expiratory flows (FEF) 25-75% were significantly reduced, whereas forced expiratory vital capacity (FVC) and FEV1/FVC were closed to normal limits, indicating a restrictive disease. All factors including; FVC, FEV1, and FEV1/FVC, FEF 25-75% were negatively correlated with age and ferritin levels. In contrast, all factors including; FVC, FEV1, and FEV1/FVC, FEF 25-75% were positively correlated with hemoglobin (Hb). We performed linear regression analysis to study the simultaneous influence of the presence of age, ferritin, and Hb on obstructive PFT indexes. Results: Pulmonary function test results were normal in only 32 (53.3%) of 60 patients and the rest 28 cases (46.7%) showed abnormal pulmonary function. FEV1 and FEF 25% - 75% have significant negative correlation with age (r = - 0.64 p(r) = 0.003 and r = - 0.58 p(r) = 0.02 respectively), also have significant positive correlation with Hb (r = 0.31 p(r) = 0.015 and r = 0.33 p(r) = 0.01 respectively), and only FEF 25% - 75% has significant negative correlation with ferritin (r -0.26 p(r) = 0.04). Conclusion: The present study has shown that restrictive disease and reduced lung diffusing capacity are the predominant abnormalities of pulmonary function patients with TM. The low hemoglobin concentration and a fall in the diffusing capacity of the alveola - capillary membrane, together with the dependence of the reduced pulmonary diffusing capacity on age and serum ferritin levels, as well as of the entity of restrictive disease on age, suggests that pulmonary

The impact of major depression on heart rate variability and endothelial dysfunction in patients with stable coronary artery disease.

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Aydin Sunbul, Esra; Sunbul, Murat; Gulec, Huseyin

Depression is an independent risk factor in cardiovascular diseases. Changes in the cardiac autonomic functions and pro-inflammatory processes are potential biological factors. Endothelial dysfunction plays an important role in the etiopathogenesis of atherosclerosis. Our objective was to evaluate the impact of major depression on heart rate variability and endothelial dysfunction in patients with stable CAD. The study group included 65 CAD patients with a diagnosis of major depression and 54 CAD patients without major depression. All study population underwent transthoracic echocardiography, measurement of flow mediated dilatation (FMD) and 24-h holter recording for heart rate variability (HRV). Blood samples were drawn to determine the inflammatory parameters. Severity of depressive episode was assessed by Montgomery-Asberg Depression Scale (MADRS). The distribution of age and sex was similar in the patient and control groups (P=0.715, 0.354, respectively). There was no significant difference in medications used between the groups. Echocardiographic parameters were similar between the groups. Inflammatory parameters were also similar between the groups. HRV parameters were significantly lower in the patient group than controls. The absolute FMD value and percentage FMD were significantly lower in the patient group than controls (Pgender (Pgender. Clinician should pay more attention for evaluation of depressive patients with CAD. Copyright © 2016 Elsevier Inc. All rights reserved.

Incidence and Risk Factors for Major Hematomas in Aesthetic Surgery: Analysis of 129,007 Patients.

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Kaoutzanis, Christodoulos; Winocour, Julian; Gupta, Varun; Ganesh Kumar, Nishant; Sarosiek, Konrad; Wormer, Blair; Tokin, Christopher; Grotting, James C; Higdon, K Kye

2017-10-16

Postoperative hematomas are one of the most frequent complications following aesthetic surgery. Identifying risk factors for hematoma has been limited by underpowered studies from single institution experiences. To examine the incidence and identify independent risk factors for postoperative hematomas following cosmetic surgery utilizing a prospective, multicenter database. A prospectively enrolled cohort of patients who underwent aesthetic surgery between 2008 and 2013 was identified from the CosmetAssure database. Primary outcome was occurrence of major hematomas requiring emergency room visit, hospital admission, or reoperation within 30 days of the index operation. Univariate and multivariate analysis was used to identify potential risk factors for hematomas including age, gender, body mass index (BMI), smoking, diabetes, type of surgical facility, procedure by body region, and combined procedures. Of 129,007 patients, 1180 (0.91%) had a major hematoma. Mean age (42.0 ± 13.0 years vs 40.9 ± 13.9 years, P hematomas. Males suffered more hematomas than females (1.4% vs 0.9%, P Hematoma rates were higher in patients undergoing combined procedures compared to single procedures (1.1% vs 0.8%, P hematoma included age (Relative Risk [RR] 1.01), male gender (RR 1.98), the procedure being performed in a hospital setting rather than an office-based setting (RR 1.68), combined procedures (RR 1.35), and breast procedures rather than the body/extremity and face procedures (RR 1.81). Major hematoma is the most common complication following aesthetic surgery. Male patients and those undergoing breast or combined procedures have a significantly higher risk of developing hematomas. 2. © 2017 The American Society for Aesthetic Plastic Surgery, Inc. Reprints and permission: journals.permissions@oup.com

Hematopoietic stem cell transplantation from non-sibling matched family donors for patients with thalassemia major in Jordan.

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Hussein, Ayad Ahmed; Al-Zaben, Abdulhadi; Khattab, Eman; Haroun, Anas; Frangoul, Haydar

2016-02-01

There are limited data on the outcome of patients with thalassemia receiving HSCT from non-sibling matched family donors. Of the 341 patients with thalassemia major that underwent donor search at our center from January 2003 to December 2011, 236 (69.2%) had fully matched family donor of which 28 patients (8.2%) had non-sibling matched family donors identified. We report on seven patients with a median age of eight yr (4-21) who underwent myeloablative (n = 4) or RIC (n = 3) HSCT. The median age of the donors was 33 yr (4-47), three were parents, two first cousins, one paternal uncle, and one paternal aunt. All patients achieved primary neutrophil and platelet engraftment at a median of 18 (13-20) and 16 days (11-20), respectively. One patient developed grade II acute GVHD, and two patients developed limited chronic GVHD. One patient experienced secondary GF requiring a second transplant. At a median follow-up of 69 months (7-110), all patients are alive and thalassemia free. Our data emphasize the need for extended family HLA typing for patients with thalassemia major in regions where there is high rate of consanguinity. Transplant from non-sibling matched family donor can result in excellent outcome. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Evaluation of cardiac and hepatic iron overload in thalassemia major patients with T2* magnetic resonance imaging.

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Wahidiyat, Pustika Amalia; Liauw, Felix; Sekarsari, Damayanti; Putriasih, Siti Ayu; Berdoukas, Vasili; Pennell, Dudley J

2017-09-01

Recent advancements have promoted the use of T2* magnetic resonance imaging (MRI) in the non-invasive detection of iron overload in various organs for thalassemia major patients. This study aims to determine the iron load in the heart and liver of patients with thalassemia major using T2* MRI and to evaluate its correlation with serum ferritin level and iron chelation therapy. This cross-sectional study included 162 subjects diagnosed with thalassemia major, who were classified into acceptable, mild, moderate, or severe cardiac and hepatic iron overload following their T2* MRI results, respectively, and these were correlated to their serum ferritin levels and iron chelation therapy. The study found that 85.2% of the subjects had normal cardiac iron stores. In contrast, 70.4% of the subjects had severe liver iron overload. A significant but weak correlation (r = -0.28) was found between cardiac T2* MRI and serum ferritin, and a slightly more significant correlation (r = 0.37) was found between liver iron concentration (LIC) and serum ferritin. The findings of this study are consistent with several other studies, which show that patients generally manifest with liver iron overload prior to cardiac iron overload. Moreover, iron accumulation demonstrated by T2* MRI results also show a significant correlation to serum ferritin levels. This is the first study of its kind conducted in Indonesia, which supports the fact that T2* MRI is undoubtedly valuable in the early detection of cardiac and hepatic iron overload in thalassemia major patients.

An ethnographic study of the effects of cognitive symptoms in patients with major depressive disorder

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Ebert, Bjarke; Miskowiak, Kamilla; Kloster, Morten

2017-01-01

BACKGROUND: The manifestation of major depressive disorder (MDD) may include cognitive symptoms that can precede the onset of MDD and persist beyond the resolution of acute depressive episodes. However, little is known about how cognitive symptoms are experienced by MDD patients and the people...... symptoms in MDD appeared to negatively impact patients' social relationships and patients' ability to handle daily tasks at work and at home; (3) patients' cognitive symptoms also impacted relationships with family members and coworkers; (4) patients' cognitive symptoms increased stress and feelings...... of failure, which in turn seemed to worsen the cognitive symptoms, thereby creating a destructive cycle; and (5) although HCPs recommended that patients re-engage in everyday activities to help overcome their depression, cognitive symptoms seemed to impede such functional recovery. CONCLUSIONS: Taken...

Persistent candidemia in major burn patients: radiologic findings of the thorax

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Lee, Eil Seong; Lee, Kwan Seop; Kang, Ik Won [Hallym Univ. College of Medicine, Seoul (Korea, Republic of)

1997-04-01

To describe radiologic findings of burn-associated persistent candidemia of the thorax. This study included 42 patients with major burns in whom blood culture had shown the presence for more than 24 hours of persistent candidemia. The duration of positive culture for candidiasis ranged from two to 67 days(mean, 15 days). Radiographic(n=42) and thin-section CT findings(n=13) were retrospectively analyzed. The onset, pattern, size, distribution and persistence of parenchymal abnormalities as well as the presence or absence of pleural effusions, mediastinal lymphadenopathy and cardiomegaly were assessed. On chest radiographs, positive findings were noticed in 61.9%(26/42) and on thin-section CT, in 76.9%(10/13). The most frequent radiographic finding was pulmonary nodule(s), observed in 14 patients(33.3%); in 13, these were bilateral. Bronchovascular bundle thickening(n=6, 14.3%), consolidation(n=4, 9.5%), cardiomegaly(n=6, 14.3%) and pleural effusion(n=4, 9.5%) were also observed. Those lesions appeared eight to 129 days(mean, 33 days) after the burn. Radiographic abnormalities persisted for seven to 115(mean, 35) days, regardless of the treatment. Thin-section CT showed parenchymal abnormalities in 10/13 patients(76.9%) and subpleural nodules of less than 1cm in diameter and without halo in all patients. Cardiomegaly, pleural effusion and mediastinal adenopathy were observed on CT in 5(38.5%), 4(30.8%) and 2(15.4%) of the 13 patients, respectively. In a high proportion of patients with burn-associated candidemia, chest radiograph and thin-section CT findings were positive. The most frequent radiographic parenchymal abnormality was multiple bilateral nodules.

Persistent candidemia in major burn patients: radiologic findings of the thorax

International Nuclear Information System (INIS)

Lee, Eil Seong; Lee, Kwan Seop; Kang, Ik Won

1997-01-01

To describe radiologic findings of burn-associated persistent candidemia of the thorax. This study included 42 patients with major burns in whom blood culture had shown the presence for more than 24 hours of persistent candidemia. The duration of positive culture for candidiasis ranged from two to 67 days(mean, 15 days). Radiographic(n=42) and thin-section CT findings(n=13) were retrospectively analyzed. The onset, pattern, size, distribution and persistence of parenchymal abnormalities as well as the presence or absence of pleural effusions, mediastinal lymphadenopathy and cardiomegaly were assessed. On chest radiographs, positive findings were noticed in 61.9%(26/42) and on thin-section CT, in 76.9%(10/13). The most frequent radiographic finding was pulmonary nodule(s), observed in 14 patients(33.3%); in 13, these were bilateral. Bronchovascular bundle thickening(n=6, 14.3%), consolidation(n=4, 9.5%), cardiomegaly(n=6, 14.3%) and pleural effusion(n=4, 9.5%) were also observed. Those lesions appeared eight to 129 days(mean, 33 days) after the burn. Radiographic abnormalities persisted for seven to 115(mean, 35) days, regardless of the treatment. Thin-section CT showed parenchymal abnormalities in 10/13 patients(76.9%) and subpleural nodules of less than 1cm in diameter and without halo in all patients. Cardiomegaly, pleural effusion and mediastinal adenopathy were observed on CT in 5(38.5%), 4(30.8%) and 2(15.4%) of the 13 patients, respectively. In a high proportion of patients with burn-associated candidemia, chest radiograph and thin-section CT findings were positive. The most frequent radiographic parenchymal abnormality was multiple bilateral nodules

Rumination mediates the relationship between overgeneral autobiographical memory and depression in patients with major depressive disorder.

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Liu, Yansong; Yu, Xinnian; Yang, Bixiu; Zhang, Fuquan; Zou, Wenhua; Na, Aiguo; Zhao, Xudong; Yin, Guangzhong

2017-03-21

Overgeneral autobiographical memory has been identified as a risk factor for the onset and maintenance of depression. However, little is known about the underlying mechanisms that might explain overgeneral autobiographical memory phenomenon in depression. The purpose of this study was to test the mediation effects of rumination on the relationship between overgeneral autobiographical memory and depressive symptoms. Specifically, the mediation effects of brooding and reflection subtypes of rumination were examined in patients with major depressive disorder. Eighty-seven patients with major depressive disorder completed the 17-item Hamilton Depression Rating Scale, Ruminative Response Scale, and Autobiographical Memory Test. Bootstrap mediation analysis for simple and multiple mediation models through the PROCESS macro was applied. Simple mediation analysis showed that rumination significantly mediated the relationship between overgeneral autobiographical memory and depression symptoms. Multiple mediation analyses showed that brooding, but not reflection, significantly mediated the relationship between overgeneral autobiographical memory and depression symptoms. Our results indicate that global rumination partly mediates the relationship between overgeneral autobiographical memory and depressive symptoms in patients with major depressive disorder. Furthermore, the present results suggest that the mediating role of rumination in the relationship between overgeneral autobiographical memory and depression is mainly due to the maladaptive brooding subtype of rumination.

Recombinant factor VIIa treatment for asymptomatic factor VII deficient patients going through major surgery.

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Livnat, Tami; Shenkman, Boris; Spectre, Galia; Tamarin, Ilia; Dardik, Rima; Israeli, Amnon; Rivkind, Avraham; Shabtai, Moshe; Marinowitz, Uri; Salomon, Ophira

2012-07-01

Factor VII deficiency is the most common among the rare autosomal recessive coagulation disorders worldwide. In factor VII deficient patients, the severity and clinical manifestations cannot be reliably determined by factor VII levels. Severe bleeding tends to occur in individuals with factor VII activity levels of 2% or less of normal. Patients with 2-10% factor VII vary between asymptomatic to severe life threatening haemorrhages behaviour. Recombinant factor VIIa (rFVIIa) is the most common replacement therapy for congenital factor VII deficiency. However, unlike haemophilia patients for whom treatment protocols are straight forward, in asymptomatic factor VII deficiency patients it is still debatable. In this study, we demonstrate that a single and very low dose of recombinant factor VIIa enabled asymptomatic patients with factor VII deficiency to go through major surgery safely. This suggestion was also supported by thrombin generation, as well as by thromboelastometry.

Stress Echocardiography and Major Cardiac Events in Patients with Normal Exercise Test

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Calasans, Flávia Ricci; Santos, Bruno Fernandes de Oliveira; Silveira, Débora Consuelo Rocha; de Araújo, Ana Carla Pereira; Melo, Luiza Dantas; Barreto-Filho, José Augusto; Sousa, Antônio Carlos Sobral; Oliveira, Joselina Luzia Menezes

2013-01-01

Background Exercise test (ET) is the preferred initial noninvasive test for the diagnosis and risk stratification of coronary artery disease (CAD), however, its lower sensitivity may fail to identify patients at greater risk of adverse events. Objective To assess the value of stress echocardiography (SE) for predicting all-cause mortality and major cardiac events (MACE) in patients with intermediate pretest probability of CAD and a normal ET. Methods 397 patients with intermediate CAD pretest probability, estimated by the Morise score, and normal ET who underwent SE were studied. The patients were divided into two groups according to the absence (G1) or presence (G2) of myocardial ischemia on SE .End points evaluated were all-cause mortality and MACE, defined as cardiac death and nonfatal acute myocardial infarction (AMI). Results G1 group was comprised of 329 (82.8%) patients. The mean age of the patients was 57.37 ± 11 years and 44.1% were male. During a mean follow-up of 75.94 ± 17.24 months, 13 patients died, three of them due to cardiac causes, and 13 patients suffered nonfatal AMI. Myocardial ischemia remained an independent predictor of MACE (HR 2.49; [CI] 95% 1.74-3.58). The independent predictors for all-cause mortality were male gender (HR 9.83; [CI] 95% 2.15-44.97) and age over 60 years (HR 4.57; [CI] 95% 1.39-15.23). Conclusion Positive SE for myocardial ischemia is a predictor of MACE in the studied sample, which helps to identify a subgroup of patients at higher risk of events despite having normal ET. PMID:23765384

Hematogenous candida pneumonia in major burn patients: plain chest radiograph and thin-section CT findings

International Nuclear Information System (INIS)

Kim, Hyo Heon; Lee, Eil Seong; Shim, Ya Seong; Kim, Ji Hun; Suh, Hong Kil; Cho, Sin Young; Kim, Dae Sun; Lee, Kil Woo; Kang, Ik Won

1995-01-01

To describe plain radiographic and thin-section CT findings of hematogenous candida pneumonia in major burn patients. We reviewed nine cases of hematogenous candida pneumonia in major burn patients who had positive blood culture for candida and findings of pneumonia on plain chest radiograph. On five of nine cases, thin-section CT was done. We evaluated retrospectively nine cases for onset, the pattern, distribution, and size of lesions on plain chest radiograph and thin-section CT. On plain chest radiograph, randomly distributed 2-10 mm nodules were seen in six cases(66%) and randomly distributed 10-15 mm consolidations in remaining three cases(33%). Lesion occurred in 11th to 75th post-burn day(average, 34th post-burn day). Other findings were cardiomegaly in three cases, atelectasis in three cases, and pulmonary edema in one case. Thin-section CT showed variable shaped subpleural nodules in all five cases. The size of nodules were 1-5 mm in two cases(40%) and 5-10 mm(60%) in three cases. Feeding vessel signs were seen in two cases. Other findings were atelectasis in three cases, cardiomegaly in three cases, ground-glass opacity and interlobular septal thickenings by pulmonary edema in two cases. Plain chest radiographic findings of hematogenous candida pneumonia in major burn patients are randomly distributed nodules or consolidations of variable size. Thin-section CT findings are variable shaped subpleural nodules less than 1 cm

Reliability of trans-abdominal ultrasonography in determining exact location of placenta in patients of placenta previa major

International Nuclear Information System (INIS)

Zafar, M.; Hayat, N; Gul, U.

2017-01-01

Objective: To determine the reliability of trans-abdominal ultrasonographical localization of placenta in cases of placenta previa major, by taking peroperative finding as gold standard. Study Design: Validation study. Place and Duration of Study: Maternity ward, Obstetrics and Gynecology department, Military Hospital Rawalpindi from 2007 to 2008. Patients and Methods: A total of 100 patients fulfilling the inclusion and exclusion criteria were recruited for the study. These patients were admitted to the maternity ward, where trans-abdominal ultrasound was performed, site of the placenta and its relation to the internal os was documented. These patients under went elective cesarean section, during which the site and relation of the placenta to the internal os was confirmed. Results: The mean age of patients was 34.23 +- 6.76 years. Transabdominal ultrasound had a sensitivity of 93.4 percent in localizing major placenta previa while the specificity was 83 percent. Positive predictive value was 94.7 percent, negative predictive value was 80 percent and accuracy 91 percent. Conclusion: Trans-abdominal ultrasound was found highly effective in diagnosing and localizing placenta previa. (author)

Morbidly obese patient with obstructive sleep apnoea for major spine surgery: An anaesthetic challenge

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Shruti Redhu

2016-01-01

Full Text Available Morbidly obese patients with clinical features of obstructive sleep apnoea can present a myriad of challenges to the anaesthesiologists which must be addressed to minimise the perioperative risks. Initiation of continuous positive airway pressure (CPAP therapy early in the pre- and post-operative period along with appropriate anaesthetic planning is of paramount importance in such patients. This case report emphasises the usefulness of CPAP therapy, even for a short duration, to minimise morbidity, improve recovery and hasten early discharge from the hospital after major surgery.

Morphological features of the major duodenal papilla in patients with cholelithiasis

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V. M. Klymenko

2017-12-01

Full Text Available The high prevalence of the pathology of major duodenal papilla in patients with gallstone disease, the difficulties of diagnosis and treatment determine the relevance of the study. The purpose is to study the morphological features of the major duodenal papilla in patients with cholelithiasis. Material and methods. During autopsies, selection of fragments of major duodenal papilla from 30 dead was performed. The main group consisted of autopsy specimens (n = 15 from the dead, which had stones in the gallbladder in the absence of signs of inflammation of the walls of the gallbladder and bile ducts. Biomaterial samples (n = 15 from the dead, which had no stones and signs of inflammation of the biliary system, were included in the control group. To study the severity of the stromal component, the Mason’s trichrome and the Van Gieson’s stains were used. Light microscopy was carried out using a light microscope Axioplan 2 (Carl Zeiss - Germany using x10, x20, x40, x100 lenses and x10 eyepiece. For a quantitative assessment of the severity of sclerotic changes, we conducted a morphometric study of preparations stained with picrofuchsin accordimg to Van Gieson. The area of the stromal component was calculated in the resulted images in 5 fields of view under magnification of x200 using the medical software for analysis and processing of digital images ImageJ, initiated by W. Rasband (1997-2012. The expression area was the percentage ratio of the number of pixels of the digital image of the zones of expression of the connective tissue component to the total number of pixels in the image. Results. The quantitative analysis of the severity of sclerotic changes in the periductal tissue in both studied groups showed that in the control observations the expression area of the connective tissue component was 18.84 ± 3.14%, and in the main group (cases of gallstone disease – 78.06 ± 15.12% (p = 0.0031. Conclusions. 1. In patients with gallstone disease

Abnormal activation of the occipital lobes during emotion picture processing in major depressive disorder patients

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Li, Jianying; Xu, Cheng; Cao, Xiaohua; Gao, Qiang; Wang, Yan; Wang, Yanfang; Peng, Juyi; Zhang, Kerang

2013-01-01

A large number of studies have demonstrated that depression patients have cognitive dysfunction. With recently developed brain functional imaging, studies have focused on changes in brain function to investigate cognitive changes. However, there is still controversy regarding abnormalities in brain functions or correlation between cognitive impairment and brain function changes. Thus, it is important to design an emotion-related task for research into brain function changes. We selected positive, neutral, and negative pictures from the International Affective Picture System. Patients with major depressive disorder were asked to judge emotion pictures. In addition, functional MRI was performed to synchronously record behavior data and imaging data. Results showed that the total correct rate for recognizing pictures was lower in patients compared with normal controls. Moreover, the consistency for recognizing pictures for depressed patients was worse than normal controls, and they frequently recognized positive pictures as negative pictures. The consistency for recognizing pictures was negatively correlated with the Hamilton Depression Rating Scale. Functional MRI suggested that the activation of some areas in the frontal lobe, temporal lobe, parietal lobe, limbic lobe, and cerebellum was enhanced, but that the activation of some areas in the frontal lobe, parietal lobe and occipital lobe was weakened while the patients were watching positive and neutral pictures compared with normal controls. The activation of some areas in the frontal lobe, temporal lobe, parietal lobe, and limbic lobe was enhanced, but the activation of some areas in the occipital lobe were weakened while the patients were watching the negative pictures compared with normal controls. These findings indicate that patients with major depressive disorder have negative cognitive disorder and extensive brain dysfunction. Thus, reduced activation of the occipital lobe may be an initiating factor for

Increased BDNF levels after electroconvulsive therapy in patients with major depressive disorder: A meta-analysis study.

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Rocha, Renan Boeira; Dondossola, Eduardo Ronconi; Grande, Antônio José; Colonetti, Tamy; Ceretta, Luciane Bisognin; Passos, Ives C; Quevedo, Joao; da Rosa, Maria Inês

2016-12-01

We performed a systematic review and meta-analysis to estimate brain-derived neurotrophic factor (BDNF) level in patients with major depressive disorder (MDD) after electroconvulsive therapy (ECT). A comprehensive search of the Cochrane Library, MEDLINE, LILACS, Grey literature, and EMBASE was performed for papers published from January 1990 to April 2016. The following key terms were searched: "major depressive disorder", "unipolar depression", "brain-derived neurotrophic factor", and "electroconvulsive therapy". A total of 252 citations were identified by the search strategy, and nine studies met the inclusion criteria of the meta-analysis. BDNF levels were increased among patients with MDD after ECT (P value = 0.006). The standardized mean difference was 0.56 (95% CI: 0.17-0.96). Additionally, we found significant heterogeneity between studies (I 2  = 73%). Our findings suggest a potential role of BDNF as a marker of treatment response after ECT in patients with MDD. Copyright © 2016 Elsevier Ltd. All rights reserved.

Radiographic appearances following adequate transfusion in. beta. -thalassaemia

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Scutellari, P.N.; Orzincolo, C.; Bagni, B.; Franceschini, F.

1989-01-01

The main lesions of the skull and hand, observed in a group of hypertransfused ..beta..-thalassaemic patients, are compared with a control group of low-transfused patients. Bony abnormalities reflect the relationship between proliferating bone marrow and bone cortex, and hypertransfusion therapy will prevent development of lesions only if established early in life. If this is done, the diploe in the skull may become normal, overgrowth of facial bones is moderate, pneumatisation of the paranasal sinuses is not completely prevented, and the 'hair-brush' pattern may disappear completely. A normal appearance of the hand in adequately treated patients differentiates between prepubertal patients and adults.

Identification of hazelnut major allergens in sensitive patients with positive double-blind, placebo-controlled food challenge results

DEFF Research Database (Denmark)

Pastorello, Elide A; Vieths, Stefan; Pravettoni, Valerio

2002-01-01

The hazelnut major allergens identified to date are an 18-kd protein homologous to Bet v 1 and a 14-kd allergen homologous to Bet v 2. No studies have reported hazelnut allergens recognized in patients with positive double-blind, placebo-controlled food challenge (DBPCFC) results or in patients...

Seroprevalence of parvovirus B19 infection in patients with beta thalassemia major in Fayoum University Hospital

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Mohamed E. Al Ghwass

2016-09-01

Conclusion: Parvovirus B19 infection is detected in high rates among children with beta thalassemia major. Measures to avoid iatrogenic and nosocomial transmission have to be implemented including screening of donated blood for B19 especially blood given to patients with hematological disorders. Also data from this study support the need for introduction of an approved B19 vaccine that primarily protects children with thalassemia major against that infection.

Major bleeding risks of different low-molecular-weight heparin agents: a cohort study in 12 934 patients treated for acute venous thrombosis.

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van Rein, N; Biedermann, J S; van der Meer, F J M; Cannegieter, S C; Wiersma, N; Vermaas, H W; Reitsma, P H; Kruip, M J H A; Lijfering, W M

2017-07-01

Essentials Low-molecular-weight-heparins (LMWH) kinetics differ which may result in different bleeding risks. A cohort of 12 934 venous thrombosis patients on LMWH was followed until major bleeding. The absolute major bleeding risk was low among patients registered at the anticoagulation clinic. Once-daily dosing was associated with a lower bleeding risk as compared with twice-daily. Background Low-molecular-weight heparins (LMWHs) are considered members of a class of drugs with similar anticoagulant properties. However, pharmacodynamics and pharmacokinetics between LMWHs differ, which may result in different bleeding risks. As these agents are used by many patients, small differences may lead to a large effect on numbers of major bleeding events. Objectives To determine major bleeding risks for different LMWH agents and dosing schedules. Methods A cohort of acute venous thrombosis patients from four anticoagulation clinics who used an LMWH and a vitamin K antagonist were followed until they ceased LMWH treatment or until major bleeding. Exposures were classified according to different types of LMWHs and for b.i.d. and o.d. use. Cumulative incidences for major bleeding per 1000 patients and risk ratios were calculated and adjusted for study center. Results The study comprised 12 934 patients with a mean age of 59 years; 6218 (48%) were men. The cumulative incidence of major bleeding was 2.5 per 1000 patients (95% confidence interval [CI], 1.7-3.5). Enoxaparin b.i.d. or o.d. was associated with a relative bleeding risk of 1.7 (95% CI, 0.2-17.5) compared with nadroparin o.d. In addition, a nadroparin b.i.d. dosing schedule was associated with a 2.0-fold increased major bleeding risk (95% CI, 0.8-5.1) as compared with a nadroparin o.d. dosing schedule. Conclusions Absolute major bleeding rates were low for all LMWH agents and dosing schedules in a large unselected cohort. Nevertheless, twice-daily dosing with nadroparin appeared to be associated with an increased

Episodic Visual Learning/Memory and Attentional Flexibility in Patients With Major Depressive Disorder After Clinically Effective Electroconvulsive Therapy.

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Kalogerakou, Stamatina; Oulis, Panagiotis; Anyfandi, Eleni; Konstantakopoulos, George; Papakosta, Vasiliki-Maria; Kontis, Dimitrios; Theochari, Eirini; Angelopoulos, Elias; Zervas, Ioannis M; Mellon, Robert C; Papageorgiou, Charalambos C; Tsaltas, Eleftheria

2015-12-01

This study is a follow-up of a previous one reporting that the neuropsychological profile of pharmacoresistant patients with major depressive disorder referred for electroconvulsive therapy (ECT, ECT group) contrasted with that of their pharmacorespondent counterparts (NECT group). The NECT group exhibited severe visuospatial memory and minor executive deficits; the ECT group presented the reverse pattern. In that same ECT group, the current follow-up study examined the effects of clinically effective ECT on both cognitive domains 2 months later. Fifteen ECT patients were administered Hamilton Depression (HAMD-24), Hamilton Anxiety (HAMA), Mini-Mental State Examination Scales and 5 tests of Cambridge Neuropsychological Test Automated Battery at intake (pre-ECT), end of ECT course (post-ECT), and 2 months thereafter (follow-up). Electroconvulsive therapy was effective in relieving clinical depression. After a post-ECT decline, the patients exhibited significant improvement in both Cambridge Neuropsychological Test Automated Battery, paired associate learning, and Stockings of Cambridge. By contrast, their major pre-ECT deficit in intra/extradimensional set shifting remained virtually unaffected. Our findings suggest that attentional flexibility deficits may constitute a neuropsychological trait-like feature of pharmacoresistant, ECT-referred major depressive disorder patients. However, this deficit does not seem generalized, given patient improvement in episodic visual learning/memory and some indication of improvement in spatial planning after ECT.

Major stroke in a 19-year-old patient with a univentricular heart

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Riemann M

2013-01-01

Full Text Available Mads Riemann,1,2 Lars Idorn,3 Aase Wagner,4 Lars Søndergaard,3 Jørgen K Kanters1,21Department of Internal Medicine, Elsinore Hospital, Elsinore, Denmark; 2Department of Biomedical Sciences, University of Copenhagen, Copenhagen, Denmark; 3Department of Cardiology, Copenhagen University Hospital, Section 2014, Rigshospitalet, Copenhagen, Denmark; 4Department of Radiology, Copenhagen University Hospital, Section 3023, Rigshospitalet, Copenhagen, DenmarkAbstract: Patients with univentricular heart malformations are at increased risk of suffering from thromboembolic events. We present a case of a 19-year-old woman born with a univentricular heart who suffered a major stroke while being treated with only salicylic acid. At least 20% of patients with univentricular hearts have been reported to experience thromboembolic events, of which 25% are fatal. Despite the high incidence of thromboembolic events, no consensus has been reached regarding the role of long-term anti-thrombotic treatment in this group of patients. This lack of consensus warrants future studies that compare the different therapeutic strategies.Keywords: univentricle, stroke, antithrombotic treatment

Goal-Directed Fluid Resuscitation Protocol Based on Arterial Waveform Analysis of Major Burn Patients in a Mass Burn Casualty.

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Chiao, Hao-Yu; Chou, Chang-Yi; Tzeng, Yuan-Sheng; Wang, Chih-Hsin; Chen, Shyi-Gen; Dai, Niann-Tzyy

2018-02-01

Adequate fluid titration during the initial resuscitation period of major burn patients is crucial. This study aimed to evaluate the feasibility and efficacy of a goal-directed fluid resuscitation protocol that used hourly urine output plus the arterial waveform analysis FloTrac (Edwards LifeSciences, Irvine, Calif) system for major burns to avoid fluid overload. We conducted a retrospective cohort study of 43 major burn patients at the Tri-Service General Hospital after the Formosa Fun Coast Dust Explosion on June 27, 2015. Because of the limited capacity of intensive care units (ICUs), 23 intubated patients were transferred from the burn wards or emergency department to the ICU within 24 hours. Fluid administration was adjusted to achieve a urine output of 30 to 50 mL/h, cardiac index greater than 2.5 L/min/m, and stroke volume variation (SVV) less than 12%. The hourly crystalloid fluid infusion rate was titrated based on SVV and hourly urine output. Of the 23 critically burned patients admitted to the ICU, 13 patients who followed the goal-directed fluid resuscitation protocol within 12 hours postburn were included in the analysis. The mean age (years) was 21.8, and the mean total body surface area (TBSA) burned (%) was 68.0. The mean Revised Baux score was 106.8. All patients sustained inhalation injury. The fluid volumes administered to patients in the first 24 hours and the second 24 hours (mL/kg/% total body surface area) were 3.62 ± 1.23 and 2.89 ± 0.79, respectively. The urine outputs in the first 24 hours and the second 24 hours (mL/kg/h) were 1.13 ± 0.66 and 1.53 ± 0.87, respectively. All patients achieved the established goals within 32 hours postburn. In-hospital mortality rate was 0%. The SVV-based goal-directed fluid resuscitation protocol leads to less unnecessary fluid administration during the early resuscitation phase. Clinicians can efficaciously manage the dynamic body fluid changes in major burn patients under the guidance of the protocol.

Evaluation of crossed cerebellar diaschisis in 30 patients with major cerebral artery occlusion by means of quantitative I-123 IMP SPECT

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Miyazawa, Nobuhiko; Toyama, Keiji; Arbab, A.S.; Arai, Takao; Nukui, Hideaki [Yamanashi Medical Univ., Tamaho (Japan); Koizumi, Kiyoshi

2001-12-01

Quantitative crossed cerebellar diaschisis (CCD) and the correlation with a reduction in supratentorial regional cerebral blood flow (rCBF) and cerebrovascular reserve capacity (CVR) were investigated in clinically stable patients with major cerebral artery occlusion by the iodine-123-N-isopropyl-p-iodoamphetamine (I-123 IMP) single photon emission computed tomography (SPECT) method. Thirty patients with major cerebral artery occlusion underwent SPECT by the I-123 IMP autoradiographic method. Regional CBF was measured in the cerebral hemisphere, frontal and parietal lobes, temporo-parietal lobe, and cerebellum both at rest and after administration of acetazolamide. Eighteen of 30 patients (60%) had CCD. CCD was significantly related to magnetic resonance imaging evidence of infarction. Quantitative CCD was 17% and the CVR in the cerebellum was preserved in patients with CCD. There was a significant difference in CBF and CVR between the affected and normal sides in all regions of interest in the patients without CCD [CBF (ml/100 g/min): hemisphere (H), normal side (N): 31.4{+-}6.8, affected side (A): 27.5{+-}7.4; p<0.05. CVR: H, N: 0.56{+-}0.38, A: 0.42{+-}0.18; p<0.01]. CCD is common in patients with major cerebral artery occlusion, and quantitative I-123 IMP SPECT is helpful in detecting CCD in clinically stable patients with occlusion of major cerebral arteries. (author)

Results of a real-world study on vortioxetine in patients with major depressive disorder in South East Asia (REVIDA).

Science.gov (United States)

Chin, Cheuk Ngen; Zain, Azhar; Hemrungrojn, Solaphat; Ung, Eng Khean; Kwansanit, Patanon; Au Yong, Koon Choong; Chong, Marvin Swee Woon; Inpa, Chalowat; Yen, Teck Hoe; Yeoh, Boon Beng David; Tay, Liam Kai; Bernardo, Carmina; Lim, Lionel Chee-Chong; Yap, Chin Hong; Fones, Calvin; Nayak, Ashwini; Nelleman, Lars

2018-05-17

The REVIDA study aimed to assess the evolution of major depression symptoms in South East Asian (SEA) patients treated with vortioxetine for major depression in real-world clinical practice. This non-interventional study was conducted from Aug 2016 to Apr 2017. A total of 138 patients (aged 18-65 years) with an active episode of major depression were recruited from Malaysia, Philippines, Singapore, and Thailand. Vortioxetine was initiated on the first visit and patients were followed for 3 months. Depression severity was assessed using the PHQ-9 questionnaire (patient-assessed) and CGI-S scale (physician-assessed); cognitive function was assessed with the PDQ-D questionnaire; work productivity and activity impairment (WPAI) was assessed with the WPAI questionnaire. At baseline, 89.9% of patients were moderately to severely depressed (PHQ-9 score ≥ 10). During the 3-month treatment period, mean±SD PHQ-9 score decreased from 18.7±5.7 to 5.0±5.3, mean±SD CGI-S score decreased from 4.4±0.7 to 2.2±1.1, and mean±SD PDQ-D score decreased from 42.1±18.8 to 13.4±13.0. By month 3, response and remission rates reached 80.8% and 59.0%, respectively. Work productivity loss decreased from 73.6% to 30.5%, while activity impairment decreased from 71.5% to 24.6%. Positive correlations were observed between PHQ-9, PDQ-D, and WPAI work productivity loss and activity impairment. By month 3, 82.0% of patients were either not depressed or only mildly depressed (PHQ-9 score ≤ 9). In real-world clinical settings, vortioxetine was effective in reducing depression severity and improving cognitive function and work productivity in SEA patients with major depression.

Depression and smoking: a 5-year prospective study of patients with major depressive disorder.

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Holma, Irina A K; Holma, K Mikael; Melartin, Tarja K; Ketokivi, Mikko; Isometsä, Erkki T

2013-06-01

Major depressive disorder (MDD) and smoking are major public health problems and epidemiologically strongly associated. However, the relationship between smoking and depression and whether this is influenced by common confounding factors remain unclear, in part due to limited longitudinal data on covariation. In the Vantaa Depression Study, psychiatric out- and inpatients with DSM-IV MDD and aged 20-59 years at were followed from baseline to 6 months, 18 months, and 5 years. We investigated course of depression, smoking, and comorbid alcohol-use disorders among the 214 patients (79.6% of 269) participating at least three time points; differences between smoking versus nonsmoking patients, and covariation of MDD, smoking, and alcohol-use disorders. Overall, 31.3% of the patients smoked regularly, 41.1% intermittently, and 27.6% never. Smokers were younger, had more alcohol-use disorders and Cluster B and C personality disorder symptoms, a higher frequency of lifetime suicide attempts, higher neuroticism, smaller social networks, and lower perceived social support than never smokers. Smoking and depression had limited longitudinal covariation. Depression, smoking, and alcohol-use disorders all exhibited strong autoregressive tendencies. Among adult psychiatric MDD patients, smoking is strongly associated with substance-use and personality disorders, which may confound research on the impact of smoking. Rather than depression or smoking covarying or predicting each other, depression, smoking, and alcohol-use disorders each have strong autoregressive tendencies. These findings are more consistent with common factors causing their association than either of the conditions strongly predisposing to the other. © 2013 Wiley Periodicals, Inc.

A comparison of functional outcome in patients sustaining major trauma: a multicentre, prospective, international study.

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Timothy H Rainer

Full Text Available OBJECTIVES: To compare 6 month and 12 month health status and functional outcomes between regional major trauma registries in Hong Kong and Victoria, Australia. SUMMARY BACKGROUND DATA: Multicentres from trauma registries in Hong Kong and the Victorian State Trauma Registry (VSTR. METHODS: Multicentre, prospective cohort study. Major trauma patients and aged ≥18 years were included. The main outcome measures were Extended Glasgow Outcome Scale (GOSE functional outcome and risk-adjusted Short-Form 12 (SF-12 health status at 6 and 12 months after injury. RESULTS: 261 cases from Hong Kong and 1955 cases from VSTR were included. Adjusting for age, sex, ISS, comorbid status, injury mechanism and GCS group, the odds of a better functional outcome for Hong Kong patients relative to Victorian patients at six months was 0.88 (95% CI: 0.66, 1.17, and at 12 months was 0.83 (95% CI: 0.60, 1.12. Adjusting for age, gender, ISS, GCS, injury mechanism and comorbid status, Hong Kong patients demonstrated comparable mean PCS-12 scores at 6-months (adjusted mean difference: 1.2, 95% CI: -1.2, 3.6 and 12-months (adjusted mean difference: -0.4, 95% CI: -3.2, 2.4 compared to Victorian patients. Keeping age, gender, ISS, GCS, injury mechanism and comorbid status, there was no difference in the MCS-12 scores of Hong Kong patients compared to Victorian patients at 6-months (adjusted mean difference: 0.4, 95% CI: -2.1, 2.8 or 12-months (adjusted mean difference: 1.8, 95% CI: -0.8, 4.5. CONCLUSION: The unadjusted analyses showed better outcomes for Victorian cases compared to Hong Kong but after adjusting for key confounders, there was no difference in 6-month or 12-month functional outcomes between the jurisdictions.

A Comparison of Functional Outcome in Patients Sustaining Major Trauma: A Multicentre, Prospective, International Study

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Rainer, Timothy H.; Yeung, Hiu Hung; Gabbe, Belinda J.; Yuen, Kai Y.; Ho, Hiu F.; Kam, Chak W.; Chang, Annice; Poon, Wai S.; Cameron, Peter A.; Graham, Colin A.

2014-01-01

Objectives To compare 6 month and 12 month health status and functional outcomes between regional major trauma registries in Hong Kong and Victoria, Australia. Summary Background Data Multicentres from trauma registries in Hong Kong and the Victorian State Trauma Registry (VSTR). Methods Multicentre, prospective cohort study. Major trauma patients and aged ≥18 years were included. The main outcome measures were Extended Glasgow Outcome Scale (GOSE) functional outcome and risk-adjusted Short-Form 12 (SF-12) health status at 6 and 12 months after injury. Results 261 cases from Hong Kong and 1955 cases from VSTR were included. Adjusting for age, sex, ISS, comorbid status, injury mechanism and GCS group, the odds of a better functional outcome for Hong Kong patients relative to Victorian patients at six months was 0.88 (95% CI: 0.66, 1.17), and at 12 months was 0.83 (95% CI: 0.60, 1.12). Adjusting for age, gender, ISS, GCS, injury mechanism and comorbid status, Hong Kong patients demonstrated comparable mean PCS-12 scores at 6-months (adjusted mean difference: 1.2, 95% CI: −1.2, 3.6) and 12-months (adjusted mean difference: −0.4, 95% CI: −3.2, 2.4) compared to Victorian patients. Keeping age, gender, ISS, GCS, injury mechanism and comorbid status, there was no difference in the MCS-12 scores of Hong Kong patients compared to Victorian patients at 6-months (adjusted mean difference: 0.4, 95% CI: −2.1, 2.8) or 12-months (adjusted mean difference: 1.8, 95% CI: −0.8, 4.5). Conclusion The unadjusted analyses showed better outcomes for Victorian cases compared to Hong Kong but after adjusting for key confounders, there was no difference in 6-month or 12-month functional outcomes between the jurisdictions. PMID:25157522

Sparing of contralateral major salivary glands has a significant effect on oral health in patients treated with radical radiotherapy of head and neck tumors

International Nuclear Information System (INIS)

Beer, K.T.; Greiner, R.H.; Zehnder, D.; Lussi, A.

2002-01-01

Background: Has a conscious exclusion of the contralateral major salivary glands (parotid, submandibular, and sublingual glands) a significant impact on the milieu of the oral cavity (saliva flow, pH, buffer capacity, and colonisation with Streptococcus mutans) in patients with ENT tumors receiving radical radiotherapy? Patients and Methods: 20 consecutive consentient patients with ENT tumors were evaluated once before, weekly during, and 6 weeks after the end of treatment in regard to saliva flow, pH, buffer capacity, and colonisation with Streptococcus mutans. In 13 patients the major salivary glands on both sides were included in the treated volume, in seven patients the treatment portals excluded consciously the contralateral major salivary glands. Results: The stimulated saliva flow decreases already during the 1st week of radiotherapy, the decrease follows the dose exponentially; the saliva flow is further reduced in the weeks after the end of treatment. The effect is less pronounced in patients with sparing of contralateral major salivary glands. The majority of patients with unilateral sparing of the major salivary glands retain the baseline value of buffer capacity, whereas buffer capacity of all patients with inclusion of all major salivary glands is markedly reduced with 20 Gy already, without signs of recovery when treatment has stopped. With unilateral salivary gland sparing the pH always remains basic, in bilaterally irradiated patients the pH changes from a mean of 7.3 to 5.8 during treatment. The colonisation with Streptococcus mutans varies little in both groups during the radiotherapy; after the end of therapy, it is higher in bilaterally irradiated patients. Conclusions: The conscious arrangement of irradiation portals in order to spare contralateral major salivary glands in patients with radical radiotherapy of ENT tumors has a significant influence on the oral environment: the stimulated saliva flow is higher, the buffer capacity retains the

The impact of chronic kidney disease as a predictor of major cardiac events in patients with no evidence of coronary artery disease

International Nuclear Information System (INIS)

Furuhashi, Tatsuhiko; Moroi, Masao; Joki, Nobuhiko; Hase, Hiroki; Masai, Hirofumi; Kunimasa, Taeko; Nakazato, Ryo; Fukuda, Hiroshi; Sugi, Kaoru

2010-01-01

Normal stress myocardial perfusion images (MPI) generally show good prognosis for cardiovascular events. However, chronic kidney disease (CKD) is one of the important risk factors for coronary artery disease (CAD), and the interpretation of normal stress MPI has not been well established in CKD patients with no evidence of CAD. The purpose of this study was to evaluate the long-term prognostic value of stress MPI in CKD patients with no evidence of myocardial ischemia or infarction. Patients who had no history but were suspected of CAD and had normal stress MPI (n=307, male=208, age=67 years, CKD/non-CKD=46/261) were followed-up for 4.5 years. CKD was defined as a glomerular filtration ratio of 2 and/or persistent proteinuria. Cardiac death, non-fatal myocardial infarction, and unstable angina requiring hospitalization were defined as major cardiac events. Major cardiac events were observed in 3 of 261 (1.1%) non-CKD patients and 6 of 46 (13%) CKD patients (p<0.001, with log-rank test). CKD was an independent risk factor for major cardiac events (hazard ratio=13.1, p<0.001, multivariate Cox regression analysis). Normal stress MPI does not always promise a good prognosis for major cardiac events. Even in patients with no evidence of CAD from stress MPI, CKD can be an independent and significant risk factor for major cardiac events. (author)

Chinese Registry of rheumatoid arthritis (CREDIT): II. prevalence and risk factors of major comorbidities in Chinese patients with rheumatoid arthritis.

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Jin, Shangyi; Li, Mengtao; Fang, Yongfei; Li, Qin; Liu, Ju; Duan, Xinwang; Liu, Yi; Wu, Rui; Shi, Xiaofei; Wang, Yongfu; Jiang, Zhenyu; Wang, Yanhong; Yu, Chen; Wang, Qian; Tian, Xinping; Zhao, Yan; Zeng, Xiaofeng

2017-11-15

Rheumatoid arthritis patients are at higher risk of developing comorbidities. The main objective of this study was to evaluate the prevalence of major comorbidities in Chinese rheumatoid arthritis patients. We also aimed to identify factors associated with these comorbidities. Baseline demographic, clinical characteristics and comorbidity data from RA patients enrolled in the Chinese Registry of rhEumatoiD arthrITis (CREDIT) from Nov 2016 to August 2017 were presented and compared with those from five other registries across the world. Possible factors related to three major comorbidities (cardiovascular disease, fragility fracture and malignancy) were identified using multivariate logistic regression analyses. A total of 13,210 RA patients were included (80.6% female, mean age 52.9 years and median RA duration 4.0 years). Baseline prevalence rates of major comorbidities were calculated: CVD, 2.2% (95% CI 2.0-2.5%); fragility fracture, 1.7% (95% CI 1.5-1.9%); malignancy, 0.6% (95% CI 0.5-0.7%); overall major comorbidities, 4.2% (95% CI 3.9-4.6%). Advanced age was associated with all comorbidities. Male gender and disease duration were positively related to CVD. Female sex and longer disease duration were potential risk factors for fragility fractures. Ever use of methotrexate (MTX) was negatively related to baseline comorbidities. Patients with rheumatoid arthritis in China have similar prevalence of comorbidities with other Asian countries. Advanced age and long disease duration are possible risk factors for comorbidities. On the contrary, MTX may protect RA patients from several major comorbidities, supporting its central role in the management of rheumatoid arthritis.

Quality of life and mortality assessment in patients with major cardiac events in the postoperative period.

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Abelha, Fernando José; Botelho, Miguela; Fernandes, Vera; Barros, Henrique

2010-01-01

Cardiovascular complications in the postoperative period are associated with high mortality and morbidity. Few studies have assessed the degree of dependence in these patients and their perception of health. The objective of this study was to assess the mortality and the quality of life in patients who developed major cardiac events (MCE) in the postoperative period. Retrospective study carried out in a Surgical Intensive Care Unit (SICU), between March 2006 and March 2008. The patients were assessed regarding the occurrence of CE. Six months after the hospital discharge, the Short-Form-36 (SF-36) questionnaire was filled out and dependence was assessed in relation to activities of daily living (ADL). The comparisons between independent groups of patients were carried out using Student's t test. The comparison between each variable and the occurrence of CE was carried out by logistic regression and included all patients. Of the 1,280 patients that met the inclusion criteria, 26 (2%) developed MCE. The univariate analysis identified as independent determinants for the development of major cardiac events: ASA physical status, hypertension, ischemic heart disease, congestive heart disease and score of the Revised Cardiac Risk Index (RCRI). The six-month mortality after the SICU discharge was 35%. Of the 17 surviving patients, 13 completed the questionnaires. Thirty-one percent of them reported that their general health was better on the day they answered the questionnaire, when compared to 12 months before. Sixty-nine percent of the patients were dependent in instrumental ADL e 15% in personal ADL. The development of MCE has a significant impact on the duration of hospital stay and mortality rates. Six months after the discharge from the SICU, more than 50% of the patients were dependent in at least one instrumental ADL. Copyright 2010 Elsevier Editora Ltda. All rights reserved.

Haemoglobinopathies in Europe: health & migration policy perspectives

OpenAIRE

Aguilar Martinez, Patricia; Angastiniotis, Michael; Eleftheriou, Androulla; Gulbis, Beatrice; Mañú Pereira, Maria Del Mar; Petrova-Benedict, Roumyana; Corrons, Joan-Lluis Vives

2014-01-01

BACKGROUND: Major haemoglobinopathies (MH), such as thalassaemia syndromes (Thal) and sickle cell disorders (SCD), are genetic defects associated with chronic anaemia and other complications. In Europe, MH are rare diseases (RD) but their prevalence is significantly growing in many countries due to mobility and migration flows. This creates a growing health problem in the EU that has not yet been effectively addressed by Member States (MS) authorities. The present study has been conducted wit...

Characteristics of patients who suffer major osteoporotic fractures despite adhering to alendronate treatment

DEFF Research Database (Denmark)

Abrahamsen, B; Rubin, K H; Eiken, Pia Agnete

2013-01-01

.03-1.06, for each drug). Dementia (HR 1.81, 95 % CI 1.18-2.78), prior fracture (one: HR 1.17, 95 % CI 1.02-1.34; multiple: HR 1.34, 95 % CI 1.08-1.67), and ulcer disease (HR 1.45, 95 % CI 1.04-2.03) also increased the risk. Diabetes did not influence fracture risk, nor did rheumatic disorders. The risk was lower...... and associations may not be causal, it may be prudent to include dementia, ulcer disease, and Parkinson's disease to capture the risk of fractures on treatment. Lower risk in patients treated with glucocorticoids and in men probably reflects a lower treatment threshold related to guidelines.......Antiresorptive treatment reduces the risk of fractures, but most patients remain at elevated risk. We used health registers to identify predictors of new major osteoporotic fractures in patients adhering to alendronate. Risk factors showed a different pattern than in the general population...

Resection and reconstruction of giant cervical metastatic cancer using a pectoralis major muscular flap transfer: A prospective study of 16 patients.

Science.gov (United States)

Zhang, Xiangmin; Liu, Folin; Lan, Xiaolin; Huang, Jing; Luo, Keqing; Li, Shaojin

2015-07-01

If not promptly or properly treated, certain cervical metastatic cancers that develop from unknown primary tumors may rapidly grow into giant tumors that can invade the blood vessels, muscle and skin. The present study examined the feasibility and efficacy of radical neck dissection combined with reconstruction using the pectoralis major myocutaneous flap for the treatment of giant cervical metastatic cancers that have developed from unknown primary tumors and have invaded the skin. A total of 16 patients who met the inclusion criteria were subjected to radical neck dissection to adequately resect invaded skin, and the pectoralis major myocutaneous flap was used to repair the large skin defect created in the cervical region. Following the surgery, the patients received concurrent chemoradiotherapy. The pectoralis major myocutaneous flap survived in all 16 patients, with no cases of flap necrosis. In addition, no post-operative lymphedema, paresthesia or dysfunction of an upper extremity occurred due to the cutting of a pectoralis major muscle. In 9 cases, patients were satisfied with their post-operative shoulder movement at the donor site; in the remaining 7 cases, patients felt greater weakness in this region following surgery relative to prior to surgery. The 14 male patients were generally satisfied with the post-operative appearance of the donor region, whereas the 2 female patients were dissatisfied with the appearance of this region. Follow-up for 6-53 months after the patients were discharged following surgery and chemotherapy revealed that the recurrence of cervical tumors in 6 cases. Overall, radical neck dissection combined with the use of the pectoralis major myocutaneous flap for reconstruction is a feasible approach for the treatment of giant cervical metastatic cancers that have developed from unknown primary tumors and have invaded the skin. Post-operative concurrent chemoradiotherapy should be administered to improve the local control rate and

Plasma glial cell line-derived neurotrophic factor in patients with major depressive disorder: a preliminary study.

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Lee, Bun-Hee; Hong, Jin-Pyo; Hwang, Jung-A; Na, Kyoung-Sae; Kim, Won-Joong; Trigo, Jose; Kim, Yong-Ku

2016-02-01

Some clinical studies have reported reduced peripheral glial cell line-derived neurotrophic factor (GDNF) level in elderly patients with major depressive disorder (MDD). We verified whether a reduction in plasma GDNF level was associated with MDD. Plasma GDNF level was measured in 23 healthy control subjects and 23 MDD patients before and after 6 weeks of treatment. Plasma GDNF level in MDD patients at baseline did not differ from that in healthy controls. Plasma GDNF in MDD patients did not differ significantly from baseline to the end of treatment. GDNF level was significantly lower in recurrent-episode MDD patients than in first-episode patients before and after treatment. Our findings revealed significantly lower plasma GDNF level in recurrent-episode MDD patients, although plasma GDNF levels in MDD patients and healthy controls did not differ significantly. The discrepancy between our study and previous studies might arise from differences in the recurrence of depression or the ages of the MDD patients.

Serum interleukin-6 is related to lower cognitive functioning in elderly patients with major depression.

Science.gov (United States)

Ali, Nehad Samir; Hashem, Abdel Hamid Hashem; Hassan, Akmal Mostafa; Saleh, Alia Adel; El-Baz, Heba Nabil

2018-05-01

There is an increased evidence of an association between inflammatory mediators, particularly serum IL-6, depression and cognitive impairment in the elderly. This study aims at exploring the relation of peripheral IL-6 to cognitive functions in elderly patients with major depressive disorder (MDD). (1) Assessment of serum IL-6 levels and cognitive functions in elderly patients suffering from major depression and comparing them to healthy age-matched control subjects; (2) correlation between serum IL-6 levels and clinical characteristics of depression and cognitive functions in these patients. The study is an observational, case-control study. It consisted of 80 subjects, 40 with the diagnosis of MDD according to the Diagnostic and Statistical Manual of Mental Disorders (DSM IV-TR) with early onset (first episode before the age of 60) and 40 community-dwelling subjects. They were subjected to the Structured Clinical Interview according to DSM-IV, Montreal Cognitive Assessment, Montgomery Asberg Depression Rating Scale, and serum IL-6 assay using ELISA. In the depression group, subjects had lower scores in cognitive testing, than the control group (p = 0.001). Serum IL-6 was found to have a negative correlation with cognitive testing in these patients even after controlling for the severity of depressive status and Body Mass Index (BMI) (p = 0.025). MDD in elderly subjects is associated with decline in cognitive functions that may be related to peripheral IL-6 levels.

Quality of life before reconstructive breast surgery: A preoperative comparison of patients with immediate, delayed, and major revision reconstruction.

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Rosson, Gedge D; Shridharani, Sachin M; Magarakis, Michael; Manahan, Michele A; Basdag, Basak; Gilson, Marta M; Pusic, Andrea L

2013-05-01

Women undergo breast reconstruction at different time-points in their cancer care; knowing patients' preoperative quality of life (QoL) is critical in the overall care of the patient with breast cancer. Our objective was to describe presurgical QoL among women undergoing immediate, delayed, or major revision breast reconstructive surgery at our institution. From March 2008 to February 2009, we administered preoperative BREAST-Q questionnaires to women who presented to our institution for breast reconstruction. Univariate and multivariate analyses were performed to compare patient cohorts across multiple QoL domains including body image, physical well-being, psychosocial well-being, and sexual well-being. Of the 231 patients who presented for preoperative consultation, 176 returned the questionnaire (response rate 76%; 117 from the immediate, 21 from the delayed, and 32 from the major revision reconstruction groups, plus 6 mixed or unknown). The three groups differed significantly (P < 0.05) across four of the six domains: body image (satisfaction with breasts), psychosocial well-being, sexual well-being, and physical well-being of the chest and upper body. The immediate reconstruction group had higher (better) scores than the delayed reconstruction group, which had higher (better) scores than the major revision group. These data suggest that women presenting for breast reconstruction at different stages of reconstruction have different baseline QoL. Such data may help us better understand patient selection, education, and expectations, and may lead to improved patient-surgeon communication. Copyright © 2013 Wiley Periodicals, Inc.

A randomized, controlled study evaluating effects of amlodipine addition to chelators to reduce iron loading in patients with thalassemia major.

Science.gov (United States)

Eghbali, Aziz; Kazemi, Hamideh; Taherahmadi, Hassan; Ghandi, Yazdan; Rafiei, Mohammad; Bagheri, Bahador

2017-12-01

Cardiomyopathy due to iron overload can be fatal in patients with thalassemia major. Calcium channel blockers seem to be effective to reduce iron loading. Our goal was to study effects of amlodipine addition to chelators on iron loading in patients with thalassemia major. This randomized, controlled, and single-center trial was performed on 56 patients with thalassemia major. Patients were randomized 1:1 to combined group (iron chelator plus amlodipine) or control group (iron chelator) for 1 year. Iron content was measured by magnetic resonance imaging; heart T2*, and liver T2*. Serum ferritin was also measured. After 12 months of treatment, myocardial T2* values had significant improvement in combined group (21.9 ± 8.0 ms to 24.5 ± 7.6 ms; P < .05); Difference between two groups was significant (P = .02). Combined treatment had no effect on hepatic T2* value (9.6 ± 2.8 ms to 9.5 ± 3.6 ms); difference between two groups was not significant (P = .2). In addition, a significant reduction was seen in serum ferritin levels in two groups. Mild gastrointestinal upset was the most common untoward effect. Addition of amlodipine to iron chelators has beneficial effects for reduction of iron loading in patients with thalassemia major. This combination therapy seems safe. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Retrievable Vena Cava Filters in Major Trauma Patients: Prevalence of Thrombus Within the Filter

International Nuclear Information System (INIS)

Mahrer, Arie; Zippel, Douglas; Garniek, Alexander; Golan, Gil; Bensaid, Paul; Simon, Daniel; Rimon, Uri

2008-01-01

The purpose of this study was to report the prevalence of thrombus within a retrievable vena cava filter inserted prophylactically in major trauma patients referred for filter extraction. Between November 2002 and August 2005, 80 retrievable inferior vena cava filters (68 Optease and 12 Gunther-Tulip) were inserted into critically injured trauma patients (mean injury severity score 33.5). The filters were inserted within 1 to 6 (mean 2) days of injury. Thirty-seven patients were referred for filter removal (32 with Optease and 5 with Gunther-Tulip). The indwelling time was 7 to 22 (mean 13) days. All patients underwent inferior vena cavography prior to filter removal. There were no insertion-related complications and all filters were successfully deployed. Forty-three (54%) of the 80 patients were not referred for filter removal, as these patients continued to have contraindications to anticoagulation. Thirty-seven patients (46%) were referred for filter removal. In eight of them (22%) a large thrombus was seen within the filters and they were left in place, all with the Optease device. The other 29 filters (36%) were removed uneventfully.We conclude that the relatively high prevalence of intrafilter thrombi with the Optease filter may be explained by either spontaneous thrombus formation or captured emboli.

Auditory evoked potentials in patients with major depressive disorder measured by Emotiv system.

Science.gov (United States)

Wang, Dongcui; Mo, Fongming; Zhang, Yangde; Yang, Chao; Liu, Jun; Chen, Zhencheng; Zhao, Jinfeng

2015-01-01

In a previous study (unpublished), Emotiv headset was validated for capturing event-related potentials (ERPs) from normal subjects. In the present follow-up study, the signal quality of Emotiv headset was tested by the accuracy rate of discriminating Major Depressive Disorder (MDD) patients from the normal subjects. ERPs of 22 MDD patients and 15 normal subjects were induced by an auditory oddball task and the amplitude of N1, N2 and P3 of ERP components were specifically analyzed. The features of ERPs were statistically investigated. It is found that Emotiv headset is capable of discriminating the abnormal N1, N2 and P3 components in MDD patients. Relief-F algorithm was applied to all features for feature selection. The selected features were then input to a linear discriminant analysis (LDA) classifier with leave-one-out cross-validation to characterize the ERP features of MDD. 127 possible combinations out of the selected 7 ERP features were classified using LDA. The best classification accuracy was achieved to be 89.66%. These results suggest that MDD patients are identifiable from normal subjects by ERPs measured by Emotiv headset.

Nice or effective? Social problem solving strategies in patients with major depressive disorder.

Science.gov (United States)

Thoma, Patrizia; Schmidt, Tobias; Juckel, Georg; Norra, Christine; Suchan, Boris

2015-08-30

Our study addressed distinct aspects of social problem solving in 28 hospitalized patients with Major Depressive Disorder (MDD) and 28 matched healthy controls. Three scenario-based tests assessed the ability to infer the mental states of story characters in difficult interpersonal situations, the capacity to freely generate good strategies for dealing with such situations and the ability to identify the best solutions among less optimal alternatives. Also, standard tests assessing attention, memory, executive function and trait empathy were administered. Compared to controls, MDD patients showed impaired interpretation of other peoples' sarcastic remarks but not of the mental states underlying other peoples' actions. Furthermore, MDD patients generated fewer strategies that were socially sensitive and practically effective at the same time or at least only socially sensitive. Overall, while the free generation of adequate strategies for difficult social situations was impaired, recognition of optimal solutions among alternatives was spared in MDD patients. Higher generation scores were associated with higher trait empathy and cognitive flexibility scores. We suggest that this specific pattern of impairments ought to be considered in the development of therapies addressing impaired social skills in MDD. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Radiology Online Patient Education Materials Provided by Major University Hospitals: Do They Conform to NIH and AMA Guidelines?

Science.gov (United States)

Prabhu, Arpan V; Donovan, Ashley L; Crihalmeanu, Tudor; Hansberry, David R; Agarwal, Nitin; Beriwal, Sushil; Kale, Hrishikesh; Heller, Matthew

The internet creates opportunities for Americans to access medical information about imaging tests and modalities to guide them in their medical decision-making. Owing to health literacy variations in the general population, the American Medical Association and National Institutes of Health recommend patient education resources to be written between the third and seventh grade levels. Our purpose is to quantitatively assess the readability levels of online radiology educational materials, written for the public, in 20 major university hospitals. In September and October 2016, we identified 20 major university hospitals with radiology residency-affiliated hospital systems. On each hospital׳s website, we downloaded all radiology-related articles written for patient use. A total of 375 articles were analyzed for readability level using 9 quantitative readability scales that are well validated in the medical literature. The 375 articles from 20 hospital systems were collectively written at an 11.4 ± 3.0 grade level (range: 8.4-17.1). Only 11 (2.9%) articles were written at the recommended third to seventh grade levels. Overall, 126 (33.6%) were written above a full high-school reading level. University of Washington Medical Center׳s articles were the most readable with a reading level corresponding to 7.9 ± 0.9. The vast majority of websites at major academic hospitals with radiology residencies designed to provide patients with information about imaging were written above the nationally recommended health literacy guidelines to meet the needs of the average American. This may limit the benefit that patients can derive from these educational materials. Copyright © 2018 Elsevier Inc. All rights reserved.

Improving major amputation rates in the multicomplex diabetic foot patient: focus on the severity of peripheral arterial disease

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Brechow, Andrej; Slesaczeck, Torsten; Münch, Dirk; Nanning, Thomas; Paetzold, Hartmut; Schwanebeck, Uta; Bornstein, Stefan

2013-01-01

Objective: Peripheral arterial disease (PAD), as well as diabetic neuropathy, is a risk factor for the development of diabetic foot ulcers. The aim of this study was to evaluate differences and predictors of outcome parameters in patients with diabetic foot by stratifying these subjects according to the severity of PAD. Research design and methods: In a prospective study, patients with new diabetic foot ulcers have been treated and investigated by structured healthcare. Subjects were recruited between 1 January 2000 and 31 December 2007. All study participants underwent a 2-year follow-up observation period. The patients underwent a standardized examination and classification of their foot ulcers according to a modification of the University of Texas Wound Classification System. The severity of PAD was estimated by measurement of the ankle brachial index (ABI) and the continuous wave Doppler flow curve into undisturbed perfusion (0.9 diabetic foot were consecutively included into the study (69% male, mean age 66.3 ± 11.0 years, mean diabetes duration 15.8 ± 10.2 years). Major amputations (above the ankle) were performed in 4.7% of the patients. 22.1% of these subjects had decompensated PAD. These subjects had delayed ulcer healing, higher risk for major amputation [odds ratio (OR) 7.7, 95% confidence interval (CI) 2.8–21.2, p diabetic foot ulcers regarding to wound healing, major amputation and mortality. PMID:23730502

Major workplace related accidents in Singapore: A major trauma centre's experience.

Science.gov (United States)

Ng, Zhi Xu; Teo, Li Tserng; Go, Karen T S; Yeo, Yen Teng; Chiu, Ming Terk

2010-12-01

Major workplace related accidents pose a significant healthcare resource challenge in Singapore. Our study looks at the epidemiology of patients who were admitted for workplace related accidents, in a single institution, with an Injury Severity Score of >9. There were 196 cases of major workplace related accidents admitted between January 2006 and December 2007. The median age of patients admitted was 37 years with a large percentage being males (95.4%) and non-residents (57.1%). The most common ethnic group was Chinese (53.1%) followed by Indians (23.5%). The most common mechanism of injury was fall from height (66.3%) followed by injuries as a result of falling objects at work (21.9%). The percentage of patients who required surgical intervention was 69.9%. Patients admitted for major workplace related accidents had a median length of stay of 5 days in the hospital, a median length of 24 days of medical leave (ML), certifying them unfit for duty and the average cost of stay for each patient was S$11,000. We have a better understanding of the epidemiology and socio-economic impact of workplace related accidents through this study. Workplace related accidents result in significant number of man-days lost from work and monetary cost to employers, medical insurance and the hospital. With an improved understanding, we propose methods to prevent and reduce such accidents in future. A direct consequence of which will be the possible reduction of hospitalisation costs and better allocation of healthcare resources in the future.

Liquid in the major incision

International Nuclear Information System (INIS)

Herrera Jaramillo, Diego Alberto; Ortega Jaramillo, Hector

2003-01-01

We present the case of a patient with spill pleural extending in the left major incision. In the chest thorax PA, we could observe one of the complex radiographic appearances that take the reconfiguration of fluid in this localization, being this appearance dependent of the patient's position. Some points are also discussed on the anatomy of the major incisions and some of their radiographic characteristics

Time to Wound Healing and Major Adverse Limb Events in Patients with Critical Limb Ischemia Treated with Endovascular Revascularization.

Science.gov (United States)

Reed, Grant W; Salehi, Negar; Giglou, Pejman R; Kafa, Rami; Malik, Umair; Maier, Michael; Shishehbor, Mehdi H

2016-10-01

There are few studies that quantify the impact of time to wound healing on outcomes after endovascular revascularization of critical limb ischemia (CLI). In this retrospective study, 179 patients with CLI and tissue loss were assessed for adverse events after endovascular therapy. Associations between time to wound healing and outcomes were determined via Cox proportional hazards analysis. The long-term probability of events was assessed with Kaplan-Meier analysis. The primary end point was major adverse limb events (MALE-major amputation, surgical endarterectomy, or bypass). Secondary end points were major amputation, need for repeat endovascular therapy, and mortality. After multivariable adjustment for time-dependent wound healing, age, renal function, diabetes, and Rutherford class, independent predictors of MALE included the presence of an unhealed wound (hazard ratio [HR], 5.2; 95% confidence interval (CI), 2.3-11.8; P wounds compared with healed wounds (log-rank P wounds healed within 4 months had a lower probability of MALE than patients who did not heal by 4 months (log-rank, P = 0.04). Unhealed wounds were also independently associated with major amputation (HR, 9.0; 95% CI, 2.6-31.1; P = 0.0004), and patients whose wounds healed by 3 months had less major amputation (log-rank, P = 0.04). Unhealed wounds were independently associated with increased risk of mortality (HR, 42.7; 95% CI, 5.7-319.0; P = 0.002) but not repeat revascularization. Unhealed wounds are an independent risk factor for MALE, major amputation, and mortality after endovascular treatment of CLI. Wound healing within 3 months is associated with less risk of major amputation, and within 4 months less risk of MALE. A focus should be on achieving wound healing as fast as possible in this population. Copyright © 2016 Elsevier Inc. All rights reserved.

Value of black blood T2* cardiovascular magnetic resonance

Directory of Open Access Journals (Sweden)

Carpenter John Paul

2011-03-01

Full Text Available Abstract Purpose To assess whether black blood T2* cardiovascular magnetic resonance is superior to conventional white blood imaging of cardiac iron in patients with thalassaemia major (TM. Materials and methods We performed both conventional white blood and black blood T2* CMR sequences in 100 TM patients to determine intra and inter-observer variability and presence of artefacts. In 23 patients, 2 separate studies of both techniques were performed to assess interstudy reproducibility. Results Cardiac T2* values ranged from 4.5 to 43.8 ms. The mean T2* values were not different between black blood and white blood acquisitions (20.5 vs 21.6 ms, p = 0.26. Compared with the conventional white blood diastolic acquisition, the coefficient of variance of the black blood CMR technique was superior for intra-observer reproducibility (1.47% vs 4.23%, p Conclusions Black blood T2* CMR has superior reproducibility and reduced imaging artefacts for the assessment of cardiac iron, in comparison with the conventional white blood technique, which make it the preferred technique for clinical practice.

Influence of family history of major depression, bipolar disorder, and suicide on clinical features in patients with major depression and bipolar disorder.

Science.gov (United States)

Serretti, Alessandro; Chiesa, Alberto; Calati, Raffaella; Linotte, Sylvie; Sentissi, Othman; Papageorgiou, Konstantinos; Kasper, Siegfried; Zohar, Joseph; De Ronchi, Diana; Mendlewicz, Julien; Amital, Daniela; Montgomery, Stuart; Souery, Daniel

2013-03-01

The extent to which a family history of mood disorders and suicide could impact on clinical features of patients suffering from major depression (MD) and bipolar disorder (BD) has received relatively little attention so far. The aim of the present work is, therefore, to assess the clinical implications of the presence of at least one first- and/or second-degree relative with a history of MD, BD and suicide in a large sample of patients with MD or BD. One thousand one hundred and fifty-seven subjects with MD and 686 subjects with BD were recruited within the context of two large projects. The impact of a family history of MD, BD, and suicide-considered both separately and together-on clinical and socio-demographic variables was investigated. A family history of MD, BD, and suicide was more common in BD patients than in MD patients. A positive family history of mood disorders and/or suicide as well as a positive family history of MD and BD separately considered, but not a positive history of suicide alone, were significantly associated with a comorbidity with several anxiety disorders and inversely associated with age of onset. The clinical implications as well as the limitations of our findings are discussed.

Quality of Life in Patients with Thalassemia Major in a Developing Country

International Nuclear Information System (INIS)

Siddiqui, S. H.; Ishtiaq, R.; Sajid, F.; Sajid, R.

2014-01-01

Objective: To determine the problems faced by thalassemic patients in their personal, psychological and social life. Study Design: A cross-sectional multi-centre survey. Place and Duration of Study: Karachi, Lahore and Quetta Centres of Fatimid Foundation, from October 2009 to October 2010. Methodology: An indigenously developed Qualifty of Life (QoL) questionnaire modified from SF-36 questionnaire was administered to 101 transfusion dependent subjects suffering from thalassemia major. Variables were analyzed using SPSS version 15 for descriptive statistics. Results: The mean age of the subjects was 10.5 years ranging from 6 - 21 years. Less than one third of the patients felt that their health was slightly worse as compared to last year. Forty five (44%) of the patients felt loneliness due to their disease. Parents of 36 (35.6%) of the children at times did not allow their children to play because of their disease. Twenty eight (27.7%) stated difficulty in mingling with children of their age. Seventy one (70.3%) of the patients reported that at some or all times they were worried about their future life and career while 70 (69.3%) admitted being taken extra care of by their friends and 56 (55.4%) by their teachers. Conclusion: The quality of life of surveyed thalassemic patients was immensely affected. Having physical impairments, social stresses, financial burdens and problems with their education and career make them very much vulnerable to psychological trauma very early in their life. All of this creates a hindrance in their way of developing into autonomous functioning adults. (author)

The Thal-index with the BTT prediction.exe to discriminate β-thalassaemia traits from other microcytic anaemias

Directory of Open Access Journals (Sweden)

Ahangama Arachchige Nilanga Nishad

2012-06-01

Full Text Available Several attempts have been made previously to differentiate β-thalassaemia trait (BTT from other microcytic anaemias using formulae with red cell (RC parameters. Presently available formulae have low sensitivity and specificity. We wanted to develop a more precise algorithm, which could be used in situations where the gold-standard test for thalassaemia diagnosis: the high performance liquid chromatography (HPLC is not available. The study was carried out prospectively from November 2008 to March 2010 from randomly collected blood samples with a mean cell volume (MCV of less than 80 fL. HbA2 measured by HPLC was used to diagnose BTT. We used Fishers stepwise linear discriminant function analysis to develop an algorithm with RC parameters. Calculated new index Thal-index was then subjected to receiver operating characteristic curve analysis to identify best cutoff to discriminate BTT from other microcytic blood films. Software was developed to predict the BTT status (BTT prediction.exe. New index, referred to as the Thal-index, was calculated using discriminant function analysis and is given as Thal-index=[(0.615xMCV +(0.518xmean corpuscular hemoglobin+ (0.446xred cell distribution width]. A value of 59 for Thal-index has 90% sensitivity and 85% specificity for differentiating BTT from other microcytic anaemias. This showed better sensitivity and specificity compared to other formulae presently used (i.e., Mentzer in Eshani, et al.. Our study gives a better answer to set-up where HPLC is not available. Although this cannot replace HPLC, BTT prediction.exe is useful to predict instantly and is the first ever computer program available for this function. 先前已利用含红细胞（RC）参数的公式做出若干尝试，以鉴别β-地中海贫血（BTT）和其他小红细胞性贫血。目前可用公式的敏感度和特异度均低。我们想要开发更精确的算法，在地中海贫�

Nonlinear analysis of EEGs of patients with major depression during different emotional states.

Science.gov (United States)

Akdemir Akar, Saime; Kara, Sadık; Agambayev, Sümeyra; Bilgiç, Vedat

2015-12-01

Although patients with major depressive disorder (MDD) have dysfunctions in cognitive behaviors and the regulation of emotions, the underlying brain dynamics of the pathophysiology are unclear. Therefore, nonlinear techniques can be used to understand the dynamic behavior of the EEG signals of MDD patients. To investigate and clarify the dynamics of MDD patients׳ brains during different emotional states, EEG recordings were analyzed using nonlinear techniques. The purpose of the present study was to assess whether there are different EEG complexities that discriminate between MDD patients and healthy controls during emotional processing. Therefore, nonlinear parameters, such as Katz fractal dimension (KFD), Higuchi fractal dimension (HFD), Shannon entropy (ShEn), Lempel-Ziv complexity (LZC) and Kolmogorov complexity (KC), were computed from the EEG signals of two groups under different experimental states: noise (negative emotional content) and music (positive emotional content) periods. First, higher complexity values were generated by MDD patients relative to controls. Significant differences were obtained in the frontal and parietal scalp locations using KFD (pemotional bias was demonstrated by their higher brain complexities during the noise period than the music stimulus. Additionally, we found that the KFD, HFD and LZC values were more sensitive in discriminating between patients and controls than the ShEn and KC measures, according to the results of ANOVA and ROC calculations. It can be concluded that the nonlinear analysis may be a useful and discriminative tool in investigating the neuro-dynamic properties of the brain in patients with MDD during emotional stimulation. Copyright © 2015 Elsevier Ltd. All rights reserved.

Posttraumatic stress disorder increases sensitivity to long term losses among patients with major depressive disorder.

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Engelmann, Jan B; Maciuba, Britta; Vaughan, Christopher; Paulus, Martin P; Dunlop, Boadie W

2013-01-01

Decisions under risk and with outcomes that are delayed in time are ubiquitous in real life and can have a significant impact on the health and wealth of the decision-maker. Despite its potential relevance for real-world choices, the degree of aberrant risky and intertemporal decision-making in patients suffering from major depressive disorder (MDD) and posttraumatic stress disorder (PTSD) has received little attention to date. We used a case-control design to compare decision-making in healthy control subjects (N=16) versus untreated depressed subjects in a current major depressive episode (N=20). In order to examine how major depressive disorder (MDD) may impact decision-making, subjects made decisions over (1) risky outcomes and (2) delayed outcomes in the domain of gains and losses using choice paradigms from neuroeconomics. In a pre-planned analysis, depressed subjects were subdivided into those with primary PTSD along with comorbid MDD (MDD+PTSD) versus those with primary MDD without PTSD (MDD-only). Choice behavior was modeled via a standard econometric model of intertemporal choice, a quasi-hyperbolic temporal discounting function, which was estimated for each subject group separately. Under conditions of potential gain, depressed subjects demonstrated greater discounting for gains across all time frames compared to controls. In the realm of losses, both subgroups of depressed subjects discounted more steeply than controls for short time frames. However, for delayed losses ranging from >1-10 years, MDD+PTSD subjects showed shallower discounting rates relative to MDD-only subjects, who continued to discount future losses steeply. Risk attitudes did not contribute to differences in intertemporal choice. Depressed patients make choices that minimize current pain and maximize current reward, despite severe later consequences or lost opportunities. Anxiety associated with PTSD may serve as a partially protective factor in decision-making about long

Imaging of serotonin transporters and its blockade by citalopram in patients with major depression using a novel SPECT ligand [123I]-ADAM

International Nuclear Information System (INIS)

Herold, N.; Amthauer, H.; Luedemann, L.; Bruhn, H.; Felix, R.; Plotkin, M.; Uebelhack, K.; Franke, L.; Uebelhack, R.

2006-01-01

We studied the midbrain SERT availability in patients with major depression and assessed the relation of SERT occupancy by citalopram to the treatment response. 21 non-medicated patients with major depression and 13 healthy controls were examined by [ 123 I]-ADAM SPECT. The midbrain SERT availability (SERT V3'') was calculated using individual MRI scans. In 13/21 patients SPECT was repeated 7 days after oral medication with citalopram (10 mg/day). We found no significant difference in the mean midbrain SERT availability between the studied patients with major depression and healthy controls (0.86 ± 0.27 vs. 0.71 ± 0.44, p = 0.069). The mean SERT occupancy accounted to 61 %. The degree of SERT blockade by citalopram did not correlate with the reduction in HAMD total score. Treatment with low-dosed citalopram caused individually variable occupancy of the midbrain-SERT and a rapid clinical improvement in 54 % of the investigated patients. (author)

Pituitary gland height evaluated by MR in patients with β-thalassemia major: a marker of pituitary gland function

International Nuclear Information System (INIS)

Argyropoulou, M.I.; Metafratzi, Z.; Efremidis, S.C.; Kiortsis, D.N.; Bitsis, S.; Tsatoulis, A.

2001-01-01

In transfusion-dependent β-thalassemia major, increased iron deposition in the pituitary gland has a cytotoxic effect leading mainly to hypogonadotropic hypogonadism. Our purpose was to assess in these patients the height of the pituitary gland and to evaluate whether it represents a marker of pituitary gland function. In 29 patients with β-thalassemia major and 35 age- and gender-matched controls the pituitary gland height was evaluated in a midline sagittal scan using a spin echo T1-weighted (500/20 TR/TE) sequence. In all patients, an extensive endocrine evaluation was performed, including measurements of spontaneous and stimulated levels of gonadotropins, thyroid hormones, growth hormone, insulin-like growth factor, and adrenal hormones. The pituitary gland height was lower in thalassemic patients with hypogonadotropic hypogonadism (n=15) (mean 3.48; SD 0.46) than in the age- and gender-matched controls (mean 6.29; SD 0.77), (P<0.001). No statistically significant difference was found between thalassemic patients without hormone dysfunction (n=14) (mean 5.34; SD 1.52) and age- and gender-matched controls (mean 5.91; SD 1.06). We conclude that in thalassemic patients the pituitary gland height is an additional marker of pituitary gland function and might be useful in clinical management. (orig.)

Pituitary gland height evaluated by MR in patients with {beta}-thalassemia major: a marker of pituitary gland function

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Argyropoulou, M.I.; Metafratzi, Z.; Efremidis, S.C. [Dept. of Radiology, Univ. of Ioannina (Greece); Kiortsis, D.N. [Dept. of Physiology, Univ. of Ioannina (Greece); Bitsis, S.; Tsatoulis, A. [Dept. of Internal Medicine, Univ. of Ioannina (Greece)

2001-12-01

In transfusion-dependent {beta}-thalassemia major, increased iron deposition in the pituitary gland has a cytotoxic effect leading mainly to hypogonadotropic hypogonadism. Our purpose was to assess in these patients the height of the pituitary gland and to evaluate whether it represents a marker of pituitary gland function. In 29 patients with {beta}-thalassemia major and 35 age- and gender-matched controls the pituitary gland height was evaluated in a midline sagittal scan using a spin echo T1-weighted (500/20 TR/TE) sequence. In all patients, an extensive endocrine evaluation was performed, including measurements of spontaneous and stimulated levels of gonadotropins, thyroid hormones, growth hormone, insulin-like growth factor, and adrenal hormones. The pituitary gland height was lower in thalassemic patients with hypogonadotropic hypogonadism (n=15) (mean 3.48; SD 0.46) than in the age- and gender-matched controls (mean 6.29; SD 0.77), (P<0.001). No statistically significant difference was found between thalassemic patients without hormone dysfunction (n=14) (mean 5.34; SD 1.52) and age- and gender-matched controls (mean 5.91; SD 1.06). We conclude that in thalassemic patients the pituitary gland height is an additional marker of pituitary gland function and might be useful in clinical management. (orig.)

The survival analysis of beta thalassemia major patients in South East of Iran

International Nuclear Information System (INIS)

Roudbari, M.; Soltani-Rad, M.; Roudbari, S.

2008-01-01

The objective was to determine the survival of beta-thalassemia major patients with transfusion, and its related factors in Southeast of Iran. This cross-sectional study was performed in Zahedan, Iran in 2007. The sample included patients who were referred from all over the Zahedan Thalassemia Center from 1998 to 2006. The data were collected using the patient's records, which were recorded by the staff during transfusion. The data included demographic and medical information blood group, blood RH, the kind of transfused blood [KTB], annual number of transfusions [ANOT], accompanied disease [AD], Hemoglobin [Hb] and ferritin level. For data analysis, the Kaplan-Meyer method, and Long Rank test together with Cox Regression were used. Forty-six of 578 patients died and 99% survived for the first year. The ages survival proportions were 5 (97.9%), 10 (97%), 15 (92.1%), and 20 (81.2%) years. The survival time showed significant relationships with the ANOT p=0.0053, KTB p=0.003, Hb=0.002 and ferritin level p=0.0087, and AD p=0.00. Using regular transfusion, paying attention to screening of transfused blood, increasing the families knowledge on the disease to prevent the bearing of thalassemia fetus, are recommended; finally, the detection and treating of the AD, are of great importance to extend the lifetime of the patients. (author)

An advance care plan decision support video before major surgery: a patient- and family-centred approach.

Science.gov (United States)

Isenberg, Sarina R; Crossnohere, Norah L; Patel, Manali I; Conca-Cheng, Alison; Bridges, John F P; Swoboda, Sandy M; Smith, Thomas J; Pawlik, Timothy M; Weiss, Matthew; Volandes, Angelo E; Schuster, Anne; Miller, Judith A; Pastorini, Carolyn; Roter, Debra L; Aslakson, Rebecca A

2018-06-01

Video-based advanc care planning (ACP) tools have been studied in varied medical contexts; however, none have been developed for patients undergoing major surgery. Using a patient- and family-centredness approach, our objective was to implement human-centred design (HCD) to develop an ACP decision support video for patients and their family members when preparing for major surgery. The study investigators partnered with surgical patients and their family members, surgeons and other health professionals to design an ACP decision support video using key HCD principles. Adapting Maguire's HCD stages from computer science to the surgical context, while also incorporating Elwyn et al 's specifications for patient-oriented decision support tool development, we used a six-stage HCD process to develop the video: (1) plan HCD process; (2) specify where video will be used; (3) specify user and organisational requirements; (4) produce and test prototypes; (5) carry out user-based assessment; (6) field test with end users. Over 450 stakeholders were engaged in the development process contributing to setting objectives, applying for funding, providing feedback on the storyboard and iterations of the decision tool video. Throughout the HCD process, stakeholders' opinions were compiled and conflicting approaches negotiated resulting in a tool that addressed stakeholders' concerns. Our patient- and family-centred approach using HCD facilitated discussion and the ability to elicit and balance sometimes competing viewpoints. The early engagement of users and stakeholders throughout the development process may help to ensure tools address the stated needs of these individuals. NCT02489799. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Comparing treatment persistence, healthcare resource utilization, and costs in adult patients with major depressive disorder treated with escitalopram or citalopram.

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Wu, Eric Q; Greenberg, Paul E; Ben-Hamadi, Rym; Yu, Andrew P; Yang, Elaine H; Erder, M Haim

2011-03-01

Major depressive disorder is the most common type of depression, affecting 6.6% of adults in the United States annually. Citalopram and escitalopram are common second-generation antidepressants used for the treatment of patients with this disorder. Because citalopram is available in generic forms that have lower acquisition costs compared with the branded escitalopram, some health plans may provide incentives to encourage the use of the generic option. Decisions based solely on drug acquisition costs may encourage the use of a therapy that is less cost-effective when treatment persistence, healthcare utilization, and overall costs are factored in. To compare, in a real-world setting, the treatment persistence, healthcare utilization, and overall costs of managing adult patients with major depressive disorder who are treated with escitalopram or citalopram. Administrative claims data (from January 1, 2003, to June 30, 2005) were analyzed for patients with major depressive disorder aged ≥18 years. Patients filled ≥1 prescriptions for citalopram or for escitalopram (first-fill time was defined as the index date) and had no second-generation antidepressant use during the 6-month preindex period. Treatment persistence, healthcare utilization, and healthcare costs were measured over the 6-month preindex and 6-month postindex periods and compared between patients treated with citalopram or escitalopram, using unadjusted and multivariate analyses. Patients receiving escitalopram (N = 10,465) were less likely to discontinue the treatment (hazard ratio 0.94; P = .005) and switch to another second-generation antidepressant (hazard ratio 0.83; P escitalopram were also less likely to have a hospital admission (odds ratio 0.88; P = .036) or an emergency department visit and had lower total healthcare costs (-$1174) and major depressive disorder-related costs (-$109; P escitalopram, patients treated with escitalopram had better treatment persistence, lower healthcare

Cardiac tamponade as a manifestation of extrapulmonary tuberculosis in β thalassemia major patient

Science.gov (United States)

Harahap, S.; Pramudita, A.; Lusiani

2018-03-01

Cardiac tamponade is a medical emergency condition. Rapid diagnosis and determination of the etiology with epidemiologic consideration may lead to earlier treatment and improved survival. Occasionally, the etiology may be clearly related to a recognized underlying disease, but the possibility of unrelated etiologies should be considered. Pericarditis tuberculosis, a rare manifestation of extrapulmonary tuberculosis in a non-HIV patient, has to be deliberate as one of the etiology, especially in the endemic area. Here, we report a case of 28 years old male with β thalassemia major presented with excessive exertion breathlessness progressing to orthopnea. Sign of cardiac tamponade was identified from echocardiography which showed large pericardial effusion with swinging heart and right atrial systolic collapse. Pericardiocentesis was performed immediately, drained 870 ml of hemorrhagic fluid from inserted pigtail. The patient was treated with the anti-tuberculosis regimen and oral corticosteroid after real-time polymerase chain reaction of Mycobacterium tuberculosis positivity in pericardial fluid. MRI T2 confirmed no haemosiderosis in patient’s heart. After treatment, the patient responded well and showed clinical improvement.

Association of obesity with cognitive function and brain structure in patients with major depressive disorder.

Science.gov (United States)

Hidese, Shinsuke; Ota, Miho; Matsuo, Junko; Ishida, Ikki; Hiraishi, Moeko; Yoshida, Sumiko; Noda, Takamasa; Sato, Noriko; Teraishi, Toshiya; Hattori, Kotaro; Kunugi, Hiroshi

2018-01-01

Obesity has been implicated in the pathophysiology of major depressive disorder (MDD), which prompted us to examine the possible association of obesity with cognitive function and brain structure in patients with MDD. Three hundred and seven patients with MDD and 294 healthy participants, matched for age, sex, ethnicity (Japanese), and handedness (right) were recruited for the study. Cognitive function was assessed using the Brief Assessment of Cognition in Schizophrenia (BACS). Gray and white matter structures were analyzed using voxel-based morphometry and diffusion tensor imaging in a subsample of patients (n = 114) whose magnetic resonance imaging (MRI) data were obtained using a 1.5 T MRI system. Verbal memory, working memory, motor speed, attention, executive function, and BACS composite scores were lower for the MDD patients than for the healthy participants (p function, and BACS composite scores were lower in obese patients (body mass index ≥ 30, n = 17) than in non-obese patients (n = 290, p left optic radiation were reduced in obese patients (n = 7) compared with non-obese patients (n = 107, p function, reduced gray matter volume, and impaired white matter integrity in cognition-related brain areas in patients with MDD. Copyright © 2017 Elsevier B.V. All rights reserved.

he effect of exercise on hippocampal volume and neurotrophines in patients with major depression–A randomized clinical trial

DEFF Research Database (Denmark)

Krogh, Jesper; Rostrup, Egill; Thomsen, Carsten

2014-01-01

BACKGROUND: The hippocampal volume is reduced in patients with major depression. Exercise leads to an increased hippocampal volume in schizophrenia and in healthy old adults. The effect of exercise on hippocampal volume is potentially mediated by brain derived neurotrophic factor (BDNF), vascular...... endothelial growth factor (VEGF), and insulin like growth factor 1 (IGF-1). The aim of this trial was to assess the effect of an aerobic exercise intervention on hippocampal volume and serum BDNF, VEGF, and IGF-1 in patients with major depression. METHODS: Patients were randomized to an aerobic exercise...... intervention (n=41) or a control condition (n=38). Both interventions consisted of three supervised sessions per week during a three months period. RESULTS: Post-intervention the increase in maximal oxygen uptake was 3.90 ml/kg/min (SD 5.1) in the aerobic exercise group and 0.95 ml/kg/min (SD 6...

Sustained major molecular response on interferon alpha-2b in two patients with polycythemia vera

DEFF Research Database (Denmark)

Larsen, Thomas Stauffer; Bjerrum, O W; Pallisgaard, N

2008-01-01

Quantitative assessment of the JAK2 V617F allele burden during disease evolution and ongoing myelosuppressive treatment is likely to be implemented in the future clinical setting. Interferon alpha has demonstrated efficacy in treatment of both chronic myeloid leukemia and the Philadelphia chromos...... with a JAK2 V617F allele burden below 1.0% in two patients with polycythemia vera treated with interferon alpha-2b (IFN-2b). Discontinuation of IFN-2b in one of the patients was followed by a sustained long-lasting (12 months of follow-up) major molecular response....

Sequential change of hemodynamic reserve in patients with major cerebral artery occlusion of severe stenosis

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Hasegawa, Y.; Yamaguchi, T.; Tsuchiya, T. (National Cardiovascular Center, Osaka (Japan). Cerebrovascular Div.); Minematsu, K. (National Cardiovascular Center, Osaka (Japan). Research Inst.); Nishimura, T. (National Cardiovascular Inst., Osaka (Japan). Dept. of Diagnostic Radiology)

1992-02-01

To identify regional vasodilatory capacity and its sequential change, we evaluated prospectively a total of 78 acetazolamide tests in 51 patients with occlusion or greater than 75% stenosis of the carotid or middle cerebral arteries. The relative distribution of cerebral blood flow was determined by single photon emission computed tomography using N-isopropyl-p-({sup 123}I)-iodoamphetamine before and after intravenous injection of acetazolamide. Reduced vasodilatory capacity was demonstrated in 20 patients (38%), including 5 patients with hemodynamic transient ischemic attacks or infarction. Follow-up acetazolamide tests revealed asymptomatic progression of the arterial lesion (from stenosis to occlusion) in 1 patient and almost complete improvement of vasodilatory capacity in 5 patients, including 3 without surgical intervention. During an average follow-up period of 18.5 months, 4 patients died from cardiac causes or neoplasm; no neurovascular events occurred. Much larger numbers of patients with longer observation periods will be necessary to clarify the contribution of chronic hemodynamic failure to subsequent stroke. However, the present data indicate that the acetazolamide test is useful for assessing the course of high grade stenosis or occlusion of major cerebral arteries. (orig.).

Current patient and healthcare worker attitudes to eHealth and the personally controlled electronic health record in major hospitals.

Science.gov (United States)

Armani, R; Mitchell, L E; Allen-Graham, J; Heriot, N R; Kotsimbos, T; Wilson, J W

2016-06-01

The current health system in Australia is comprised of both electronic- and paper-based medical records. The Federal Government has approved funding for the development of an individual health identifier and a universally adopted online health repository. To determine attitudes and beliefs of patients and healthcare workers regarding the use of stored medical information and the personally controlled electronic health record (PCEHR) in selected major hospitals in Victoria. Qualitative survey of patients and healthcare workers (n = 600 each group) conducted during 2014 across five major hospitals in Melbourne to measure the awareness, attitudes and barriers to electronic health and the PCEHR. Of the patients, 93.3% support the concept of a shared electronic healthcare record, 33.7% were aware of the PCEHR and only 11% had registered. The majority of healthcare workers believed that the presence of a shared health record would result in an increased appropriateness of care and patient safety by reducing adverse drug events and improving the timeliness of care provided. However, only 46% of healthcare workers were aware of the PCEHR. This study provides a baseline evaluation of perceptions surrounding eHealth and PCHER in acute health services in five metropolitan centres. While there appears to be a readiness for adoption of these strategies for healthcare documentation, patients require motivation to register for the PCEHR, and healthcare workers require more information on the potential benefits to them to achieve more timely and efficient care. © 2016 Royal Australasian College of Physicians.

The major brain endocannabinoid 2-AG controls neuropathic pain and mechanical hyperalgesia in patients with neuromyelitis optica.

Directory of Open Access Journals (Sweden)

Hannah L Pellkofer

Full Text Available Recurrent myelitis is one of the predominant characteristics in patients with neuromyelitis optica (NMO. While paresis, visual loss, sensory deficits, and bladder dysfunction are well known symptoms in NMO patients, pain has been recognized only recently as another key symptom of the disease. Although spinal cord inflammation is a defining aspect of neuromyelitis, there is an almost complete lack of data on altered somatosensory function, including pain. Therefore, eleven consecutive patients with NMO were investigated regarding the presence and clinical characteristics of pain. All patients were examined clinically as well as by Quantitative Sensory Testing (QST following the protocol of the German Research Network on Neuropathic Pain (DFNS. Additionally, plasma endocannabinoid levels and signs of chronic stress and depression were determined. Almost all patients (10/11 suffered from NMO-associated neuropathic pain for the last three months, and 8 out of 11 patients indicated relevant pain at the time of examination. Symptoms of neuropathic pain were reported in the vast majority of patients with NMO. Psychological testing revealed signs of marked depression. Compared to age and gender-matched healthy controls, QST revealed pronounced mechanical and thermal sensory loss, strongly correlated to ongoing pain suggesting the presence of deafferentation-induced neuropathic pain. Thermal hyperalgesia correlated to MRI-verified signs of spinal cord lesion. Heat hyperalgesia was highly correlated to the time since last relapse of NMO. Patients with NMO exhibited significant mechanical and thermal dysesthesia, namely dynamic mechanical allodynia and paradoxical heat sensation. Moreover, they presented frequently with either abnormal mechanical hypoalgesia or hyperalgesia, which depended significantly on plasma levels of the endogenous cannabinoid 2-arachidonoylglycerole (2-AG. These data emphasize the high prevalence of neuropathic pain and hyperalgesia

Definition of major bleeding in clinical investigations of antihemostatic medicinal products in surgical patients

DEFF Research Database (Denmark)

Schulman, S; Angerås, U; Bergqvist, D

2010-01-01

subcommittee on Control of Anticoagulation, of the International Society on Thrombosis and Haemostasis has previously published a recommendation for a harmonized definition of major bleeding in non-surgical studies. That definition has been adopted by the European Medicines Agency and is currently used......The definition of major bleeding varies between studies on surgical patients, particularly regarding the criteria for surgical wound-related bleeding. This diversity contributes to the difficulties in comparing data between trials. The Scientific and Standardization Committee (SSC), through its...... in several non-surgical trials. A preliminary proposal for a parallel definition for surgical studies was presented at the 54(th) Annual Meeting of the SSC in Vienna, July 2008. Based on those discussions and further consultations with European and North American surgeons with experience from clinical trials...

Outcome and quality of life of patients with acute kidney injury after major surgery.

Science.gov (United States)

Abelha, F J; Botelho, M; Fernandes, V; Barros, H

2009-01-01

In postoperative critically-ill patients who develop Acute Kidney Injury (AKI) it is important to focus on survival and quality of life beyond hospital discharge. The aim of the study was to evaluate outcome and quality of life in patients that develop AKI after major surgery. This retrospective study was carried out in a Post-Anaesthesia Care Unit with five intensive care beds during 2 years. Patients were followed for the development of AKI. Preoperative characteristics, intra-operative management and outcome were evaluated. Six months after discharge, these patients were contacted to complete a Short Form-36 questionnaire (SF-36) and to have their dependency in ADL evaluated. Chi-square or Fischer's exact test were used to compare proportions between groups. A "t test" and a paired "t test" for independent groups was used for comparisons. Of 1584 patients admitted to the PACU, 1200 patients met the inclusion criteria. One hundred-fourteen patients (9.6%) met AKI criteria. Patients with AKI were more severely ill, stayed longer at the PACU. Among 71 hospital survivors at 6 months follow-up, 50 completed the questionnaires. Fifty-two percent of patients reported that their general level of health was better on the day they answered the questionnaire than 12 months earlier. Patients that met AKI criteria after surgery had worse SF-36 scores for physical function, role physical and role emotional domains. Six months after PACU discharge, patients that met AKI criteria were more dependent in I-ADL but not in P-ADL. Patients that develop AKI improved self-perception of quality of life despite having high rate of dependency in ADL tasks. For physical function and role physical domains they had worse scores than PACU patients that did not develop AKI.

Anterograde Amnesia during Electroconvulsive Therapy: A Prospective Pilot-Study in Patients with Major Depressive Disorder.

Directory of Open Access Journals (Sweden)

Elvira Boere

Full Text Available Electroconvulsive therapy (ECT is considered an effective treatment for major depression with melancholic features. However, neurocognitive side-effects such as anterograde amnesia still regularly occur. The present study aims to evaluate the severity and course of anterograde amnesia in severely depressed patients undergoing ECT. In a prospective naturalistic study, anterograde memory function was assessed among inpatients who underwent ECT (n = 11. Subjects met DSM-IV criteria for major depressive disorder. Recruitment took place between March 2010-March 2011 and March 2012-March 2013. Controls treated with antidepressants (n = 9 were matched for age, gender and depression severity. Primary outcome measure was immediate recall; secondary outcome measures were delayed recall, recognition, and visual association. Differences were tested using repeated measures ANOVA and paired t-tests. Correlations with hypothesized covariates were calculated. In patients with major depressive disorder, ECT had a significant effect on delayed memory function (p<0.01 with large effect sizes. Findings on immediate recall were less consistent. Four weeks after treatment discontinuation, these memory functions had recovered. Age was identified as a very important covariate. The main limitations of our study are its naturalistic design, possibly compromising internal validity, and its small sample size. However, if these findings can be reproduced in a more comprehensive study group, then the possible induction of anterograde amnesia is not a justifiable reason for clinicians to disregard ECT as a treatment option.

Mismatch negativity of sad syllables is absent in patients with major depressive disorder.

Science.gov (United States)

Pang, Xiaomei; Xu, Jing; Chang, Yi; Tang, Di; Zheng, Ya; Liu, Yanhua; Sun, Yiming

2014-01-01

Major depressive disorder (MDD) is an important and highly prevalent mental disorder characterized by anhedonia and a lack of interest in everyday activities. Additionally, patients with MDD appear to have deficits in various cognitive abilities. Although a number of studies investigating the central auditory processing of low-level sound features in patients with MDD have demonstrated that this population exhibits impairments in automatic processing, the influence of emotional voice processing has yet to be addressed. To explore the automatic processing of emotional prosodies in patients with MDD, we analyzed the ability to detect automatic changes using event-related potentials (ERPs). This study included 18 patients with MDD and 22 age- and sex-matched healthy controls. Subjects were instructed to watch a silent movie but to ignore the afferent acoustic emotional prosodies presented to both ears while continuous electroencephalographic activity was synchronously recorded. Prosodies included meaningless syllables, such as "dada" spoken with happy, angry, sad, or neutral tones. The mean amplitudes of the ERPs elicited by emotional stimuli and the peak latency of the emotional differential waveforms were analyzed. The sad MMN was absent in patients with MDD, whereas the happy and angry MMN components were similar across groups. The abnormal sad emotional MMN component was not significantly correlated with the HRSD-17 and HAMA scores, respectively. The data indicate that patients with MDD are impaired in their ability to automatically process sad prosody, whereas their ability to process happy and angry prosodies remains normal. The dysfunctional sad emotion-related MMN in patients with MDD were not correlated with depression symptoms. The blunted MMN of sad prosodies could be considered a trait of MDD.

A break-even analysis of major ear surgery.

Science.gov (United States)

Wasson, J D; Phillips, J S

2015-10-01

To determine variables which affect cost and profit for major ear surgery and perform a break-even analysis. Retrospective financial analysis. UK teaching hospital. Patients who underwent major ear surgery under general anaesthesia performed by the senior author in main theatre over a 2-year period between dates of 07 September 2010 and 07 September 2012. Income, cost and profit for each major ear patient spell. Variables that affect major ear surgery profitability. Seventy-six patients met inclusion criteria. Wide variation in earnings, with a median net loss of £-1345.50 was observed. Income was relatively uniform across all patient spells; however, theatre time of major ear surgery at a cost of £953.24 per hour varied between patients and was the main determinant of cost and profit for the patient spell. Bivariate linear regression of earnings on theatre time identified 94% of variation in earnings was due to variation in theatre time (r = -0.969; P break-even time for major ear surgery of 110.6 min. Theatre time was dependent on complexity of procedure and number of OPCS4 procedures performed, with a significant increase in theatre time when three or more procedures were performed during major ear surgery (P = 0.015). For major ear surgery to either break-even or return a profit, total theatre time should not exceed 110 min and 36 s. © 2015 John Wiley & Sons Ltd.

The ICET-A Recommendations for the Diagnosis and Management of Disturbances of Glucose Homeostasis in Thalassemia Major Patients

Science.gov (United States)

De Sanctis, Vincenzo; Soliman, Ashraf T.; Elsedfy, Heba; Yaarubi, Saif AL; Skordis, Nicos; Khater, Doaa; El Kholy, Mohamed; Stoeva, Iva; Fiscina, Bernadette; Angastiniotis, Michael; Daar, Shahina; Kattamis, Christos

2016-01-01

Iron overload in patients with thalassemia major (TM) affects glucose regulation and is mediated by several mechanisms. The pathogenesis of glycaemic abnormalities in TM is complex and multifactorial. It has been predominantly attributed to a combination of reduced insulin secretory capacity and insulin resistance. The exact mechanisms responsible for progression from norm glycaemia to overt diabetes in these patients are still poorly understood but are attributed mainly to insulin deficiency resulting from the toxic effects of iron deposited in the pancreas and insulin resistance. A group of endocrinologists, haematologists and paediatricians, members of the International Network of Clinicians for Endocrinopathies in Thalassemia and Adolescence Medicine (ICET-A) convened to formulate recommendations for the diagnosis and management of abnormalities of glucose homeostasis in thalassemia major patients on the basis of available evidence from clinical and laboratory data and consensus practice. The results of their work and discussions are described in this article. PMID:27872738

Light therapy for better mood and insulin sensitivity in patients with major depression and type 2 diabetes: a randomised, double-blind, parallel-arm trial

NARCIS (Netherlands)

Brouwer, A.; van Raalte, D.H.; Diamant, M.; Rutters, F.; van Someren, E.J.W.; Snoek, F.J.; Beekman, A.T.F.; Bremmer, M.A.

2015-01-01

Background: Major depression and type 2 diabetes often co-occur. Novel treatment strategies for depression in type 2 diabetes patients are warranted, as depression in type 2 diabetes patients is associated with poor prognosis and treatment results. Major depression and concurrent sleep disorders

Light therapy for better mood and insulin sensitivity in patients with major depression and type 2 diabetes : a randomised, double-blind, parallel-arm trial

NARCIS (Netherlands)

Brouwer, Annelies; van Raalte, Daniël H; Diamant, Michaela; Rutters, Femke; van Someren, Eus J W; Snoek, Frank J; Beekman, Aartjan T F; Bremmer, Marijke A

2015-01-01

BACKGROUND: Major depression and type 2 diabetes often co-occur. Novel treatment strategies for depression in type 2 diabetes patients are warranted, as depression in type 2 diabetes patients is associated with poor prognosis and treatment results. Major depression and concurrent sleep disorders

Brain functional changes in facial expression recognition in patients with major depressive disorder before and after antidepressant treatment

OpenAIRE

Jiang, Wenyan; Yin, Zhongmin; Pang, Yixin; Wu, Feng; Kong, Lingtao; Xu, Ke

2012-01-01

Functional magnetic resonance imaging was used during emotion recognition to identify changes in functional brain activation in 21 first-episode, treatment-naive major depressive disorder patients before and after antidepressant treatment. Following escitalopram oxalate treatment, patients exhibited decreased activation in bilateral precentral gyrus, bilateral middle frontal gyrus, left middle temporal gyrus, bilateral postcentral gyrus, left cingulate and right parahippocampal gyrus, and inc...

Major cost savings associated with biologic dose reduction in patients with inflammatory arthritis.

LENUS (Irish Health Repository)

Murphy, C L

2015-01-01

The purpose of this study was to explore whether patients with Inflammatory Arthritis (IA) (Rheumatoid Arthritis (RA), Psoriatic Arthritis (PsA) or Ankylosing Spondylitis (AS)) would remain in remission following a reduction in biologic dosing frequency and to calculate the cost savings associated with dose reduction. This prospective non-blinded non-randomised study commenced in 2010. Patients with Inflammatory Arthritis being treated with a biologic agent were screened for disease activity. A cohort of those in remission according to standardized disease activity indices (DAS28 < 2.6, BASDAI < 4) was offered a reduction in dosing frequency of two commonly used biologic therapies (etanercept 50 mg once per fortnight instead of weekly, adalimumab 40 mg once per month instead of fortnightly). Patients were assessed for disease activity at 3, 6, 12, 18 and 24 months following reduction in dosing frequency. Cost saving was calculated. 79 patients with inflammatory arthritis in remission were recruited. 57% had rheumatoid arthritis (n = 45), 13% psoriatic arthritis (n = 10) and 30% ankylosing spondylitis (n = 24). 57% (n = 45) were taking etanercept and 43% (n = 34) adalimumab. The percentage of patients in remission at 24 months was 56% (n = 44). This resulted in an actual saving to the state of approximately 600,000 euro over two years. This study demonstrates the reduction in biologic dosing frequency is feasible in Inflammatory Arthritis. There was a considerable cost saving at two years. The potential for major cost savings in biologic usage should be pursued further.

Effect of preoperative angina pectoris on cardiac outcomes in patients with previous myocardial infarction undergoing major noncardiac surgery (data from ACS-NSQIP).

Science.gov (United States)

Pandey, Ambarish; Sood, Akshay; Sammon, Jesse D; Abdollah, Firas; Gupta, Ena; Golwala, Harsh; Bardia, Amit; Kibel, Adam S; Menon, Mani; Trinh, Quoc-Dien

2015-04-15

The impact of preoperative stable angina pectoris on postoperative cardiovascular outcomes in patients with previous myocardial infarction (MI) who underwent major noncardiac surgery is not well studied. We studied patients with previous MI who underwent elective major noncardiac surgeries within the American College of Surgeons-National Surgical Quality Improvement Program (2005 to 2011). Primary outcome was occurrence of an adverse cardiac event (MI and/or cardiac arrest). Multivariable logistic regression models evaluated the impact of stable angina on outcomes. Of 1,568 patients (median age 70 years; 35% women) with previous MI who underwent major noncardiac surgery, 5.5% had postoperative MI and/or cardiac arrest. Patients with history of preoperative angina had significantly greater incidence of primary outcome compared to those without anginal symptoms (8.4% vs 5%, p = 0.035). In secondary outcomes, reintervention rates (22.5% vs 11%, p angina. In multivariable analyses, preoperative angina was a significant predictor for postoperative MI (odds ratio 2.49 [1.20 to 5.58]) and reintervention (odds ratio 2.40 [1.44 to 3.82]). In conclusion, our study indicates that preoperative angina is an independent predictor for adverse outcomes in patients with previous MI who underwent major noncardiac surgery, and cautions against overreliance on predictive tools, for example, the Revised Cardiac Risk Index, in these patients, which does not treat stable angina and previous MI as independent risk factors during risk prognostication. Copyright © 2015 Elsevier Inc. All rights reserved.

Health-related quality of life and symptom severity in Chinese patients with major depressive disorder.

Science.gov (United States)

Cao, Yuping; Li, Wen; Shen, Jingjin; Malison, Robert T; Zhang, Yalin; Luo, Xingguang

2013-12-01

Patients suffering from major depressive disorder (MDD) have been reported to have substantial long-lasting limitations in multiple domains of health-related quality of life (HRQoL). The thoughtful assessment of HRQoL and the impact of treatment response on HRQoL are emerging as important issues in the care of patients with major depressive disorder. One hundred and three patients meeting Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) criteria for MDD took fluoxetine (20 mg/d) for 6 weeks and were assessed by the Short Form 36 Health Survey (SF-36), the 17-item Hamilton Depression Rating (HAMD-17) and the Clinical Global Impression (CGI) scales. Relationships between SF-36 scores and depressive symptom severity and early change of these symptoms were tested. SF-36 component scores at week 6 were higher than those at baseline (all P ≤ 0.0058). Scores for general health were significantly higher in responders than non-responders (P = 0.0009). The overall HAMD-17 and CGI scores at 2- and 6-week follow-up were significantly lower than those at baseline (P ≤ 0.0001). Higher scores for anxiety/somatization were significantly associated with poorer SF-36 scores at baseline (P = 0.0001); role-physical scores at week 6 were positively correlated with reduction rate of anxiety/somatization in 2-week follow-up (P = 0.0002). Depressive symptom severity was associated with HRQoL in patients with MDD. HRQoL may vary with severity of depression and/or anxiety-somatization at baseline. Copyright © 2013 Wiley Publishing Asia Pty Ltd.

Readability assessment of patient education materials on major otolaryngology association websites.

Science.gov (United States)

Eloy, Jean Anderson; Li, Shawn; Kasabwala, Khushabu; Agarwal, Nitin; Hansberry, David R; Baredes, Soly; Setzen, Michael

2012-11-01

Various otolaryngology associations provide Internet-based patient education material (IPEM) to the general public. However, this information may be written above the fourth- to sixth-grade reading level recommended by the American Medical Association (AMA) and National Institutes of Health (NIH). The purpose of this study was to assess the readability of otolaryngology-related IPEMs on various otolaryngology association websites and to determine whether they are above the recommended reading level for patient education materials. Analysis of patient education materials from 9 major otolaryngology association websites. The readability of 262 otolaryngology-related IPEMs was assessed with 8 numerical and 2 graphical readability tools. Averages were evaluated against national recommendations and between each source using analysis of variance (ANOVA) with post hoc Tukey's honestly significant difference (HSD) analysis. Mean readability scores for each otolaryngology association website were compared. Mean website readability scores using Flesch Reading Ease test, Flesch-Kincaid Grade Level, Coleman-Liau Index, SMOG grading, Gunning Fog Index, New Dale-Chall Readability Formula, FORCAST Formula, New Fog Count Test, Raygor Readability Estimate, and the Fry Readability Graph ranged from 20.0 to 57.8, 9.7 to 17.1, 10.7 to 15.9, 11.6 to 18.2, 10.9 to 15.0, 8.6 to 16.0, 10.4 to 12.1, 8.5 to 11.8, 10.5 to 17.0, and 10.0 to 17.0, respectively. ANOVA results indicate a significant difference (P < .05) between the websites for each individual assessment. The IPEMs found on all otolaryngology association websites exceed the recommended fourth- to sixth-grade reading level.

Brain activation predicts treatment improvement in patients with major depressive disorder.

LENUS (Irish Health Repository)

Samson, Andrea C

2012-02-01

Major depressive disorder (MDD) is associated with alterations in brain function that might be useful for therapy evaluation. The current study aimed to identify predictors for therapy improvement and to track functional brain changes during therapy. Twenty-one drug-free patients with MDD underwent functional MRI twice during performance of an emotional perception task: once before and once after 4 weeks of antidepressant treatment (mirtazapine or venlafaxine). Twelve healthy controls were investigated once with the same methods. A significant difference between groups was a relative greater activation of the right dorsolateral prefrontal cortex (dlPFC) in the patients vs. controls. Before treatment, patients responding better to pharmacological treatment showed greater activation in the dorsomedial PFC (dmPFC), posterior cingulate cortex (pCC) and superior frontal gyrus (SFG) when viewing of negative emotional pictures was compared with the resting condition. Activations in the caudate nucleus and insula contrasted for emotional compared to neutral stimuli were also associated with successful treatment. Responders had also significantly higher levels of activation, compared to non-responders, in a range of other brain regions. Brain activation related to treatment success might be related to altered self-referential processes and a differential response to external emotional stimuli, suggesting differences in the processing of emotionally salient stimuli between those who are likely to respond to pharmacological treatment and those who will not. The present investigation suggests the pCC, dmPFC, SFG, caudate nucleus and insula may have a key role as a biological marker for treatment response and predictor for therapeutic success.

A multi-institutional analysis of 429 patients undergoing major hepatectomy for colorectal cancer liver metastases: The impact of concomitant bile duct resection on survival.

Science.gov (United States)

Postlewait, Lauren M; Squires, Malcolm H; Kooby, David A; Weber, Sharon M; Scoggins, Charles R; Cardona, Kenneth; Cho, Clifford S; Martin, Robert C G; Winslow, Emily R; Maithel, Shishir K

2015-10-01

Data are lacking on long-term outcomes of patients undergoing major hepatectomy requiring bile duct resection (BDR) for the treatment of colorectal cancer liver metastases. Patients who underwent major hepatectomy (≥3 segments) for metastatic colorectal cancer from 2000-2010 at three US academic institutions were included. The primary outcome was disease-specific survival (DSS). Of 429 patients, nine (2.1%) underwent BDR, which was associated with pre-operative portal vein embolization (25.0% vs. 4.3%; P = 0.049). There were no significant differences in age, ASA class, margin status, number of lesions, tumor size, cirrhosis, perineural invasion, or lymphovascular invasion. BDR was independently associated with increased postoperative major complications (OR: 6.22; 95%CI:1.44-26.97; P = 0.015). There were no differences in length of stay, reoperation, readmission, or 30-day mortality. Patients who underwent BDR had markedly decreased DSS (9.3 vs. 39.9 mo; P = 0.002). When accounting for differences between the two groups, the need for BDR was independently associated with reduced DSS (HR: 3.06; 95%CI:1.12-8.34; P = 0.029). Major hepatectomy with concomitant bile duct resection is seldom performed in patients undergoing resection of colorectal cancer liver metastases and is associated with higher major morbidity and reduced disease-specific survival compared to major hepatectomy alone. Stringent selection criteria should be applied when patients may need bile duct resection during hepatectomy for colorectal cancer liver metastases. © 2015 Wiley Periodicals, Inc.

Cognitive-behavioral group therapy is an effective treatment for major depression in hemodialysis patients.

Science.gov (United States)

Duarte, Priscila Silveira; Miyazaki, Maria Cristina; Blay, Sergio Luís; Sesso, Ricardo

2009-08-01

Depression is an important target of psychological assessment in patients with end-stage renal disease because it predicts their morbidity, mortality, and quality of life. We assessed the effectiveness of cognitive-behavioral therapy in chronic hemodialysis patients diagnosed with major depression by the Mini International Neuropsychiatric Interview (MINI). In a randomized trial conducted in Brazil, an intervention group of 41 patients was given 12 weekly sessions of cognitive-behavioral group therapy led by a trained psychologist over 3 months while a control group of 44 patients received the usual treatment offered in the dialysis unit. In both groups, the Beck Depression Inventory, the MINI, and the Kidney Disease and Quality of Life-Short Form questionnaires were administered at baseline, after 3 months of intervention or usual treatment, and after 9 months of follow-up. The intervention group had significant improvements, compared to the control group, in the average scores of the Beck Depression Inventory overall scale, MINI scores, and in quality-of-life dimensions that included the burden of renal disease, sleep, quality of social interaction, overall health, and the mental component summary. We conclude that cognitive-behavioral group therapy is an effective treatment of depression in chronic hemodialysis patients.

Light therapy for better mood and insulin sensitivity in patients with major depression and type 2 diabetes: a randomised, double-blind, parallel-arm trial

NARCIS (Netherlands)

Brouwer, Annelies; van Raalte, Daniël H.; Diamant, Michaela; Rutters, Femke; van Someren, Eus J. W.; Snoek, Frank J.; Beekman, Aartjan T. F.; Bremmer, Marijke A.

2015-01-01

Major depression and type 2 diabetes often co-occur. Novel treatment strategies for depression in type 2 diabetes patients are warranted, as depression in type 2 diabetes patients is associated with poor prognosis and treatment results. Major depression and concurrent sleep disorders have been

Dynamic Change of Total Bilirubin after Portal Vein Embolization is Predictive of Major Complications and Posthepatectomy Mortality in Patients with Hilar Cholangiocarcinoma.

Science.gov (United States)

Ou Yang, Qing; Zhang, Sheng; Cheng, Qing-Bao; Li, Bin; Feng, Fei-Ling; Yu, Yong; Luo, Xiang-Ji; Lin, Zhao-Fen; Jiang, Xiao-Qing

2016-05-01

This study aims to evaluate the role of dynamic change in total bilirubin after portal vein embolization (PVE) in predicting major complications and 30-day mortality in patients with hilar cholangiocarcinoma (HCCA). Retrospective analysis of prospectively maintained data of 64 HCCA patients who underwent PVE before hepatectomy in our institution was used. Total bilirubin and other parameters were measured daily in peri-PVE period. The difference between them and the baseline value from days 0-5 to day -1 (∆D1) and days 5-14 to day -1 (∆D2) were calculated. The relationship between ∆D1 and ∆D2 of total bilirubin and major complications as well as 30-day mortality was analyzed. Out of 64 patients, 10 developed major complications (15.6 %) and 6 patients (9.3 %) had died within 30 days after surgery. The ∆D2 of total bilirubin after PVE was most significantly associated with major complications (P 3 (OR = 12.048; 95 % CI 1.019-143.321), ∆D2 of total bilirubin (OR = 1.058; 95 % CI 1.007-1.112), and ∆D2 of prealbumin (OR = 0.975; 95 % CI 0.952-0.999) were associated with higher risk of 30-day mortality after PVE. Receiver operating characteristic curves showed that ∆D2 of total bilirubin were better predictors than ∆D1 for major complications (AUC (∆D2) 0.817; P = 0.002 vs. AUC (∆D1) 0.769; P = 0.007) and 30-day mortality (ACU(∆D2) 0.868; P = 0.003 vs. AUC(∆D1) 0.721;P = 0.076). Patients with increased total bilirubin in 5-14 days after PVE may indicate a higher risk of major complications and 30-day mortality if the major hepatectomy were performed.

The role of major depression in neurocognitive functioning in patients with posttraumatic stress disorder

Directory of Open Access Journals (Sweden)

Mirjam J. Nijdam

2013-04-01

Full Text Available Background: Posttraumatic stress disorder (PTSD and major depressive disorder (MDD frequently co-occur after traumatic experiences and share neurocognitive disturbances in verbal memory and executive functioning. However, few attempts have been made to systematically assess the role of a comorbid MDD diagnosis in neuropsychological studies in PTSD. Objective: The purpose of the current study is to investigate neurocognitive deficits in PTSD patients with and without MDD. We hypothesized that PTSD patients with comorbid MDD (PTSD+MDD would have significantly lower performance on measures of verbal memory and executive functioning than PTSD patients without MDD (PTSD–MDD. Method: Participants included in this study were 140 treatment-seeking outpatients who had a diagnosis of PTSD after various single traumatic events and participated in a randomized controlled trial comparing different treatment types. Baseline neuropsychological data were compared between patients with PTSD+MDD (n=84 and patients with PTSD–MDD (n=56. Results: The PTSD+MDD patients had more severe verbal memory deficits in learning and retrieving words than patients with PTSD alone. There were no differences between the groups in recall of a coherent paragraph, recognition, shifting of attention, and cognitive interference. Conclusions: The results of this study suggest that a more impaired neurocognitive profile may be associated with the presence of comorbid MDD, with medium-sized group differences for verbal memory but not for executive functioning. From a clinical standpoint, being aware that certain verbal memory functions are more restricted in patients with comorbid PTSD and MDD may be relevant for treatment outcome of trauma-focused psychotherapy.

Vitamin D, parathyroid hormone, serum calcium and phosphorus in patients with schizophrenia and major depression.

Science.gov (United States)

Jamilian, Hamidreza; Bagherzadeh, Kamran; Nazeri, Zeinab; Hassanijirdehi, Marzieh

2013-02-01

Vitamin D deficiency has been associated with an increased risk of depression and schizophrenia. The aim was to compare serum levels of vitamin D, calcium, phosphorus and parathyroid hormone in schizophrenics, depressed patients and healthy subjects in an Iranian population. In a cross-sectional study, 100 patients with schizophrenia and 100 with major depression were enrolled. A questionnaire was filled by using medical records of patients. After that a serum sample was taken and levels of vitamin D, calcium, phosphorus and parathyroid hormone were assessed and then compared between the three groups. Post-hoc analysis of Tukey showed that vitamin D level in healthy participants was significantly higher than depressed patients and schizophrenics while there was no significant difference between vitamin D level in depressed and schizophrenic patients. The findings suggest that vitamin D affects the brain independent of hormonal pathways which regulate serum level of calcium. Non-significant difference in the serum level of vitamin D between the schizophrenics and the depressed patients suggests that the independent effect of vitamin D in brain is a general effect and is not specialized to a specific region or pathway in the brain; however, differences between psychiatric and non-psychiatric patients might be resulted from differences in psychosocial backgrounds.

Component resolution reveals additional major allergens in patients with honeybee venom allergy.

Science.gov (United States)

Köhler, Julian; Blank, Simon; Müller, Sabine; Bantleon, Frank; Frick, Marcel; Huss-Marp, Johannes; Lidholm, Jonas; Spillner, Edzard; Jakob, Thilo

2014-05-01

Detection of IgE to recombinant Hymenoptera venom allergens has been suggested to improve the diagnostic precision in Hymenoptera venom allergy. However, the frequency of sensitization to the only available recombinant honeybee venom (HBV) allergen, rApi m 1, in patients with HBV allergy is limited, suggesting that additional HBV allergens might be of relevance. We performed an analysis of sensitization profiles of patients with HBV allergy to a panel of HBV allergens. Diagnosis of HBV allergy (n = 144) was based on history, skin test results, and allergen-specific IgE levels to HBV. IgE reactivity to 6 HBV allergens devoid of cross-reactive carbohydrate determinants (CCD) was analyzed by ImmunoCAP. IgE reactivity to rApi m 1, rApi m 2, rApi m 3, nApi m 4, rApi m 5, and rApi m 10 was detected in 72.2%, 47.9%, 50.0%, 22.9%, 58.3%, and 61.8% of the patients with HBV allergy, respectively. Positive results to at least 1 HBV allergen were detected in 94.4%. IgE reactivity to Api m 3, Api m 10, or both was detected in 68.0% and represented the only HBV allergen-specific IgE in 5% of the patients. Limited inhibition of IgE binding by therapeutic HBV and limited induction of Api m 3- and Api m 10-specific IgG4 in patients obtaining immunotherapy supports recent reports on the underrepresentation of these allergens in therapeutic HBV preparations. Analysis of a panel of CCD-free HBV allergens improved diagnostic sensitivity compared with use of rApi m 1 alone, identified additional major allergens, and revealed sensitizations to allergens that have been reported to be absent or underrepresented in therapeutic HBV preparations. Copyright © 2014 The Authors. Published by Mosby, Inc. All rights reserved.

Performance monitoring and empathy during active and observational learning in patients with major depression.

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Thoma, Patrizia; Norra, Christine; Juckel, Georg; Suchan, Boris; Bellebaum, Christian

2015-07-01

Previous literature established a link between major depressive disorder (MDD) and altered reward processing as well as between empathy and (observational) reward learning. The aim of the present study was to assess the effects of MDD on the electrophysiological correlates - the feedback-related negativity (FRN) and the P300 - of active and observational reward processing and to relate them to trait cognitive and affective empathy. Eighteen patients with MDD and 16 healthy controls performed an active and an observational probabilistic reward-learning task while event- related potentials were recorded. Also, participants were assessed with regard to self-reported cognitive and affective trait empathy. Relative to healthy controls, patients with MDD showed overall impaired learning and attenuated FRN amplitudes, irrespective of feedback valence and learning type (active vs. observational), but comparable P300 amplitudes. In the patient group, but not in controls, higher trait perspective taking scores were significantly correlated with reduced FRN amplitudes. The pattern of results suggests impaired prediction error processing and a negative effect of higher trait empathy on feedback-based learning in patients with MDD. Copyright © 2015 Elsevier B.V. All rights reserved.

Differential diagnosis of bipolar disorder and major depressive disorder.

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Hirschfeld, R M

2014-12-01

Patients with bipolar disorder spend approximately half of their lives symptomatic and the majority of that time suffering from symptoms of depression, which complicates the accurate diagnosis of bipolar disorder. Challenges in the differential diagnosis of bipolar disorder and major depressive disorder are reviewed, and the clinical utility of several screening instruments is evaluated. The estimated lifetime prevalence of major depressive disorder (i.e., unipolar depression) is over 3 and one-half times that of bipolar spectrum disorders. The clinical presentation of a major depressive episode in a bipolar disorder patient does not differ substantially from that of a patient with major depressive disorder (unipolar depression). Therefore, it is not surprising that without proper screening and comprehensive evaluation many patients with bipolar disorder may be misdiagnosed with major depressive disorder (unipolar depression). In general, antidepressants have demonstrated little or no efficacy for depressive episodes associated with bipolar disorder, and treatment guidelines recommend using antidepressants only as an adjunct to mood stabilizers for patients with bipolar disorder. Thus, correct identification of bipolar disorder among patients who present with depression is critical for providing appropriate treatment and improving patient outcomes. Clinical characteristics indicative of bipolar disorder versus major depressive disorder identified in this review are based on group differences and may not apply to each individual patient. The overview of demographic and clinical characteristics provided by this review may help medical professionals distinguish between major depressive disorder and bipolar disorder. Several validated, easily administered screening instruments are available and can greatly improve the recognition of bipolar disorder in patients with depression. Copyright © 2014 Elsevier B.V. All rights reserved.

Deficits of magnetoencephalography regional power in patients with major depressive disorder:an individual spectral analysis

Institute of Scientific and Technical Information of China (English)

汤浩

2014-01-01

Objective To explore the discrepancies of individualized frequency and band power between major depressive disorder(MDD)and controls in resting state,and the association of abnormal spectral power with clinical severity of MDD.Methods Whole-head MEG recordings were collected in 19 patients with MDD and 19 non-depressed controls in eye-closed resting state.Individual spectral power of each subject was calculated based on

Differences in serotonin transporter binding affinity in patients with major depressive disorder and night eating syndrome.

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Lundgren, J D; Amsterdam, J; Newberg, A; Allison, K C; Wintering, N; Stunkard, A J

2009-03-01

We examined serotonin transporter (SERT) binding affinity using single photon emission computed tomography (SPECT) in patients with major depressive disorder (MDD) and night eating syndrome (NES). There are similarities between MDD and NES in affective symptoms, appetite disturbance, nighttime awakenings, and, particularly, response to selective serotonin reuptake inhibitors (SSRIs). Six non-depressed patients with NES and seven patients with MDD underwent SPECT brain imaging with 123I-ADAM, a radiopharmaceutical agent selective for SERT sites. Uptake ratios of 123I-ADAM SERT binding were obtained for the midbrain, basal ganglia, and temporal lobe regions compared to the cerebellum reference region. Patients with NES had significantly greater SERT uptake ratios (effect size range 0.64-0.84) in the midbrain, right temporal lobe, and left temporal lobe regions than those with MDD whom we had previously studied. Pathophysiological differences in SERT uptake between patients with NES and MDD suggest these are distinct clinical syndromes.

Posttraumatic stress disorder increases sensitivity to long term losses among patients with major depressive disorder.

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Jan B Engelmann

Full Text Available BACKGROUND: Decisions under risk and with outcomes that are delayed in time are ubiquitous in real life and can have a significant impact on the health and wealth of the decision-maker. Despite its potential relevance for real-world choices, the degree of aberrant risky and intertemporal decision-making in patients suffering from major depressive disorder (MDD and posttraumatic stress disorder (PTSD has received little attention to date. METHOD: We used a case-control design to compare decision-making in healthy control subjects (N=16 versus untreated depressed subjects in a current major depressive episode (N=20. In order to examine how major depressive disorder (MDD may impact decision-making, subjects made decisions over (1 risky outcomes and (2 delayed outcomes in the domain of gains and losses using choice paradigms from neuroeconomics. In a pre-planned analysis, depressed subjects were subdivided into those with primary PTSD along with comorbid MDD (MDD+PTSD versus those with primary MDD without PTSD (MDD-only. Choice behavior was modeled via a standard econometric model of intertemporal choice, a quasi-hyperbolic temporal discounting function, which was estimated for each subject group separately. RESULTS: Under conditions of potential gain, depressed subjects demonstrated greater discounting for gains across all time frames compared to controls. In the realm of losses, both subgroups of depressed subjects discounted more steeply than controls for short time frames. However, for delayed losses ranging from >1-10 years, MDD+PTSD subjects showed shallower discounting rates relative to MDD-only subjects, who continued to discount future losses steeply. Risk attitudes did not contribute to differences in intertemporal choice. CONCLUSIONS: Depressed patients make choices that minimize current pain and maximize current reward, despite severe later consequences or lost opportunities. Anxiety associated with PTSD may serve as a partially

Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries: A 15-Year Experience With 458 Patients.

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Bauser-Heaton, Holly; Borquez, Alejandro; Han, Brian; Ladd, Michael; Asija, Ritu; Downey, Laura; Koth, Andrew; Algaze, Claudia A; Wise-Faberowski, Lisa; Perry, Stanton B; Shin, Andrew; Peng, Lynn F; Hanley, Frank L; McElhinney, Doff B

2017-04-01

Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction. We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure. Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries. © 2017 American Heart Association, Inc.

Clinical, immunological and genetic features in eleven Algerian patients with major histocompatibility complex class II expression deficiency

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Djidjik Réda

2012-08-01

Full Text Available Abstract Presenting processed antigens to CD4+ lymphocytes during the immune response involves major histocompatibility complex class II molecules. MHC class II genes transcription is regulated by four transcription factors: CIITA, RFXANK, RFX5 and RFXAP. Defects in these factors result in major histocompatibility complex class II expression deficiency, a primary combined immunodeficiency frequent in North Africa. Autosomal recessive mutations in the RFXANK gene have been reported as being the principal defect found in North African patients with this disorder. In this paper, we describe clinical, immunological and genetic features of 11 unrelated Algerian patients whose monocytes display a total absence of MHC class II molecules. They shared mainly the same clinical picture which included protracted diarrhoea and respiratory tract recurrent infections. Genetic analysis revealed that 9 of the 11 patients had the same RFXANK founder mutation, a 26 bp deletion (named I5E6-25_I5E6+1, also known as 752delG26. Immunological and genetic findings in our series may facilitate genetic counselling implementation for Algerian consanguineous families. Further studies need to be conducted to determine 752delG26 heterozygous mutation frequency in Algerian population.

Effectiveness of trauma team on medical resource utilization and quality of care for patients with major trauma.

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Wang, Chih-Jung; Yen, Shu-Ting; Huang, Shih-Fang; Hsu, Su-Chen; Ying, Jeremy C; Shan, Yan-Shen

2017-07-24

Trauma is one of the leading causes of death in Taiwan, and its medical expenditure escalated drastically. This study aimed to explore the effectiveness of trauma team, which was established in September 2010, on medical resource utilization and quality of care among major trauma patients. This was a retrospective study, using trauma registry data bank and inpatient medical service charge databases. Study subjects were major trauma patients admitted to a medical center in Tainan during 2009 and 2013, and was divided into case group (from January, 2011 to August, 2013) and comparison group (from January, 2009 to August, 2010). Significant reductions in several items of medical resource utilization were identified after the establishment of trauma team. In the sub-group of patients who survived to discharge, examination, radiology and operation charges declined significantly. The radiation and examination charges reduced significantly in the subcategories of ISS = 16 ~ 24 and ISS > 24 respectively. However, no significant effectiveness on quality of care was identified. The establishment of trauma team is effective in containing medical resource utilization. In order to verify the effectiveness on quality of care, extended time frame and extra study subjects are needed.

The correlates of stigma toward mental illness among Jordanian patients with major depressive disorder.

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Rayan, Ahmad; Mahroum, Maryam Husnee; Khasawneh, Aws

2018-04-01

This study aims to assess the correlates of stigma toward mental illness among patients diagnosed with major depressive disorder (MDD). One hundred and sixty one Jordanian outpatients suffering from MDD completed the study. Participants completed the demographic questionnaire, the Center for Epidemiological Studies for the intensity of depression, and the Devaluation-Discrimination Scale to assess stigma. Participants reported a moderate level of perceived stigma toward mental illness. Age, perceived pain, the number of relapses, and severity of depressive symptoms were significantly correlated with stigma toward mental illness among the study sample. The severity of depressive symptoms was the strongest correlate of stigma toward mental illness. Factors associated with stigma toward mental illness should be carefully considered when implementing anti-stigma programs for patients. © 2017 Wiley Periodicals, Inc.

Gender differences in a cohort of major depressive patients: further evidence for the male depression syndrome hypothesis.

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Azorin, Jean-Michel; Belzeaux, Raoul; Fakra, Eric; Kaladjian, Arthur; Hantouche, Elie; Lancrenon, Sylvie; Adida, Marc

2014-01-01

Previous studies have shown that major depressive patients may differ in several features according to gender, but the existence of a specific male depressive syndrome remains controversial. As part of the EPIDEP National Multisite French Study of 493 consecutive DSM-IV major depressive patients evaluated in at least two semi-structured interviews 1 month apart, 125 (27.7%) were of male gender, whereas 317 (72.3%) were female, after exclusion of bipolar I patients. Compared to women, men were more often married, had more associated mixed features, with more bipolar disorder NOS, more hyperthymic temperaments, and less depressive temperaments. Women had an earlier age at onset of depression, more depressive episodes and suicide attempts. A higher family loading was shown in men for bipolar disorder, alcohol use disorder, impulse control disorders and suicide, whereas their family loading for major depressive disorder was lower. Men displayed more comorbidities with alcohol use, impulse control, and cardiovascular disorders, with lower comorbidities with eating, anxiety and endocrine/metabolic disorders. The following independent variables were associated with male gender: hyperthymic temperament (+), alcohol use disorder (+), impulse control disorders (+), and depressive temperament (-). The retrospective design and the lack of specific tools to assess the male depressive syndrome. Study findings may lend support to the male depression syndrome concept and draw attention to the role of hyperthymic temperament, soft bipolarity as well as comorbidities as determinants of this syndrome. The latter could help recognize an entity which is probably underdiagnosed, but conveys a high risk of suicide and cardiovascular morbidity. Copyright © 2014 Elsevier B.V. All rights reserved.

The efficacy of group metacognitive therapy on self-esteem and mental health of patients suffering from major depressive disorder.

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Farahmand, Vahid; Hassanzadeh, Ramezan; Mirzaian, Bahram; Fayyazi Bordbar, Mohammad Reza; Feizi, Jaleh

2014-01-01

The present research aims to analyze the efficacy of group metacognitive therapy (MCT) on self-esteem and mental Health of those who suffer from major depressive disorder. The research was a randomized clinical controlled trial, using pretest and posttest with 2 months of follow-up. Twenty-two patients with major depressive disorder based on DSM-IV-TR criteria were selected through available sampling from patients of two psychiatric hospitals of Mashhad, Iran, in 2011. They were allocated randomly into two groups of trial (n = 11) and control (n = 11). Citalopram and sertraline were prescribed as antidepressant to both groups. The experimental group also attended nine 90-minute sessions of MCT (a 5-week program). Eysenck self-esteem scale (ESES) and Mental Health Checklist (MHC) were used in pretest, posttest, and follow-up as the study instrument. The data were analyzed by analysis of covariance (ANCOVA) using SPSS. ANCOVA revealed that the patients receiving group MCT had significantly increased (p self-esteem in posttest, which remained significant in the follow up (p self-steam and recuperation of mental health in MDD patients.

A screening algorithm for early detection of major depressive disorder in head and neck cancer patients post-treatment: Longitudinal study.

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Henry, Melissa; Rosberger, Zeev; Ianovski, Lola E; Hier, Michael; Zeitouni, Anthony; Kost, Karen; Mlynarek, Alex; Black, Martin; MacDonald, Christina; Richardson, Keith; Zhang, Xun; Fuhrmann, Fabienne; Chartier, Gabrielle; Frenkiel, Saul

2018-03-13

The primary purpose of this study was to identify predictors of Major Depressive Disorder in head and neck cancer (HNC) patients in the immediate post-treatment period (ie, at 3 months post-diagnosis), with a focus on previously unexamined historical and contextual factors. Prospective longitudinal study of 223 consecutive adults (72% participation) newly diagnosed with a first occurrence of primary HNC, including validated psychometric measures, Structured Clinical Interviews for DSM Disorders, and medical chart reviews. The 3-month period prevalence of Major Depressive Disorder was 20.4%; with point prevalences of 6.8% upon HNC diagnosis, 14.2% at 3 months, and 22.6% lifetime. Patients most susceptible to developing Major Depressive Disorder in the immediate post-treatment period: were diagnosed with advanced-stage cancer rather than early-stage cancer (O.R. = 4.94, P = 0.04), received surgery only (O.R. = 8.73, P = 0.04), presented a lifetime history of Anxiety Disorder on SCID-I (O.R. = 6.62; P = 0.01), and indicated higher pre-treatment levels of anxiety on the HADS (O.R. = 0.45, P = 0.05). Our results outline the predominant role of anxiety upon diagnosis as a precursor to post-treatment Major Depressive Disorder, suggesting the need for identification and prophylactic treatment of anxiety upon diagnosis in head and neck cancer patients. Further investigation into pathways by which pre-treatment anxiety predisposes to post-treatment Major Depressive Disorder in this population is warranted. Copyright © 2018 John Wiley & Sons, Ltd.

Odour recognition memory and odour identification in patients with mild and severe major depressive disorders.

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Zucco, Gesualdo M; Bollini, Fabiola

2011-12-30

Olfactory deficits, in detection, recognition and identification of odorants have been documented in ageing and in several neurodegenerative and psychiatric conditions. However, olfactory abilities in Major Depressive Disorder (MDD) have been less investigated, and available studies have provided inconsistent results. The present study assessed odour recognition memory and odour identification in two groups of 12 mild MDD patients (M age 41.3, range 25-57) and 12 severe MDD patients (M age, 41.9, range 23-58) diagnosed according to DSM-IV criteria and matched for age and gender to 12 healthy normal controls. The suitability of olfactory identification and recognition memory tasks as predictors of the progression of MDD was also addressed. Data analyses revealed that Severe MDD patients performed significantly worse than Mild MDD patients and Normal controls on both tasks, with these last groups not differing significantly from one another. The present outcomes are consistent with previous studies in other domains which have shown reliable, although not conclusive, impairments in cognitive function, including memory, in patients with MDD, and highlight the role of olfactory identification and recognition tasks as an important additional tool to discriminate between patients characterised by different levels of severity of MDD. Copyright © 2011 Elsevier Ltd. All rights reserved.

Stressful life events preceding the onset of depression in Asian patients with major depressive disorder.

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Park, Subin; Hatim, Ahmad; Si, Tian-Mei; Jeon, Hong Jin; Srisurapanont, Manit; Bautista, Dianne; Liu, Shen-ing; Chua, Hong Choon; Hong, Jin Pyo

2015-12-01

Previous studies have identified the significant role of stressful life events in the onset of depressive episodes. However, there is a paucity of cross-national studies on stressful life events that precede depression. We aimed to compare types of stressful life events associated with the onset of depressive episodes in patients with major depressive disorder (MDD) in five Asian countries. A total of 507 outpatients with MDD were recruited in China (n = 114), South Korea (n = 101), Malaysia (n = 90), Thailand (n = 103) and Taiwan (n = 99). All patients were assessed with the Mini-International Neuropsychiatric Interview and the List of Threatening Experiences. The prevalence of each type of stressful life events was calculated and compared between each country. The type of stressful life event that preceded the onset of a depressive episode differed between patients in China and Taiwan and those in South Korea, Malaysia and Thailand. Patients in China and Taiwan were less likely to report interpersonal relationship problems and occupational/financial problems than patients in South Korea, Malaysia and Thailand. Understanding the nature and basis of culturally determined susceptibilities to specific stressful life events is critical for establishing a policy of depression prevention and providing effective counseling services for depressed patients. © The Author(s) 2015.

Factors associated with failure to achieve remission and with relapse after remission in patients with major depressive disorder in the PERFORM study.