Risk Factors for Pulmonary Hypertension In Patients with Thalassemia Intermedia

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 2069-2069
Author(s):  
Mehran Karimi ◽  
Khaled M Musallam ◽  
Maria Domenica Cappellini ◽  
Amal El-Beshlawy ◽  
Khawla Belhoul ◽  
...  

Abstract Abstract 2069 Introduction: Mechanisms leading to pulmonary hypertension (PHT) in patients with thalassemia intermedia (TI) are still controversial. Moreover, clinical and laboratory characteristics of patients who eventually develop PHT have not been identified. Our aim was to identify factors that characterize TI patients who develop PHT. Methods: Data was retrospectively retrieved from the Thalassemia Intermedia Registry, a database of 584 TI patients currently registered at six comprehensive care centers in Lebanon, Italy, Iran, Egypt, United Arab Emirates, and Oman. Institutional review boards at each center approved the study protocol. Two Groups of patients were identified: Group I, TI patients with documented PHT (n=64; mean age 37.3 ± 10.6; 44% males); and Group II, age- and sex-matched TI patients without PHT (n=64; mean age 37.9 ± 11.4; 44% males). Collected data included demographics, laboratory parameters, disease-complications, and received treatments that may influence PHT development and reflected the period prior to PHT occurrence. Results: There were no statistically significant differences in mean platelet counts, total or fetal hemoglobin levels between the two Groups. However, mean serum ferritin level was higher in Group I compared to Group II (1233.2 ± 499.2 vs. 654.7 ± 234.5 ng/ml; P=0.01). Moreover, mean nucleated red blood cell (NRBC) count was higher in Group I compared to Group II (354.2 ± 199.2 vs. 214.7 ± 94.5 ×10⋀6/l; P=0.03). A higher proportion of patients were splenectomized (84.4% vs. 46.9%; P<0.001) or had a previous history of thromboembolic events (40.6% vs. 7.8%; P<0.001) in Group I compared to Group II. Conversely, a higher proportion of patients received transfusion (78.1% vs. 56.2%; P<0.001), iron chelation (62.5% vs. 37.5%; P<0.001), or hydroxycarbamide (34.4% vs. 17.2%; P<0.001) therapy in Group II compared to Group I. There were no statistically significant differences in the proportion of patients with heart failure, prothrombotic mutations, or receiving antiplatelet or anticoagulant therapy between the two Groups. Multivariate logistic regression analysis revealed that patients in Group I are more likely to be splenectomized (OR:4.9, 95%CI:1.9-8.5); transfusion-naive (OR:3.5, 95%CI:2.1-6.25); on no hydroxycarbamide (OR:2.6, 95%CI:1.1-5.25) or iron chelation therapy (OR:2.3, 95%CI:1.2-4.25); and have NRBC count >300 ×10⋀6/l (OR:2.59, 95%CI:1.69-6.05) or a previous history of thromboembolism (OR:3.69, 95%CI:2.38-7.05). Conclusion: Splenectomy, previous history of thromboembolism, and a high NRBC count characterize TI patients who develop PHT. Transfusion, iron chelation, and hydroxycarbamide therapy deserve evaluation for a protective role against PHT in TI. Disclosures: No relevant conflicts of interest to declare.

2020 ◽  
Vol 78 (5) ◽  
pp. 247-254
Author(s):  
Senay AYDIN ◽  
Cengiz ÖZDEMIR ◽  
Ayşegül GÜNDÜZ ◽  
Meral E. KIZILTAN

ABSTRACT Objective: Seizures are a neurological condition commonly experienced during the follow-up period after systemic or metabolic disorders. The aim of the present study was to determine the etiological factors of seizures in patients at a tertiary care chest clinic. Methods: We reviewed all neurology consultations that were requested due to seizures in inpatient clinics in a tertiary care hospital specializing in respiratory disorders between January 2011 and January 2018 were retrospectively reviewed. Results: The present study included 705 of 2793 (25.2%) patients who requested consultations for seizures during the study period. The mean age of the sample was 64.05±17.19 years. Of the 705 patients, 307 (43.5%) had a previous history of epilepsy (Group I) and 398 (56.5%) had a first-time seizure and were considered to have symptomatic seizures (Group II). Multiple factors played roles in the development of seizures in 54.8% of the patients. In most patients, metabolic causes, systemic infections, and drug use were identified and an intracranial metastatic mass lesion was the major cause in patients with lung cancer. Rates of hypoxemia and respiratory acidosis were significantly higher in patients with symptomatic seizures (Group II) than in patients with primary epilepsy (Group I). Conclusions: Blood gas changes such as hypoxemia and respiratory acidosis were among the factors statistically associated with the development of symptomatic seizures in patients with respiratory diseases. Additionally, hypoxemia, hypercapnia, and respiratory acidosis were correlated with mortality in patients hospitalized for respiratory system diseases who requested consultations for seizures.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3260-3260
Author(s):  
Yasser Wali ◽  
A. Hakim Al-Rawas ◽  
Shoaib Al Zadjali ◽  
Murtadha K. Al-Khabori ◽  
Wafa Bashir ◽  
...  

Abstract Abstract 3260 Background: The standard of care for patients with thalassemia intermedia (TI) is regular follow up and iron chelation therapy. Patients with TI are usually transfusion independent and receive irregular transfusion when they develop low hemoglobin levels as in case of infection. However, with the current policy, these patients are liable to many complications; thalassemic facies, growth retardation, splenomegaly, hypersplenism, hypercoagulability, pulmonary hypertension, heart failure, cholelithiasis, diabetes mellitus, hypothyroidism, osteoporosis, and hypogonadism. We planned to investigate the impact of early introduction of hypertransfusion on delaying these complications. Material and Methods: All newly diagnosed children with TI at the Pediatric Thalassemia Day Care Centre, Sultan Qaboos University Hospital, Oman, starting from August 2006 were included in the study. They were diagnosed as intermedia based on clinical grounds of late age presentation (>5 years), maintaining mean baseline hemoglobin of 7 g/dL (range 6.1–7.9 g/dL), and transfusion independence. In addition, eight patients had a definitive TI mutation Hb Dhofar [β29(GGC–GGT)gly-gly, β58(CCT–CGT)pro-arg]. They were 10 females and 10 males included in the study. The mean age at presentation was 7.17 ± 3.9 years. At diagnosis, they were started on monthly hypertransfusion program to maintain a pretransfusion hemoglobin level above 9.5 g/dl and a post transfusion level of 14 g/dl. The mean duration of transfusion therapy was 6.2 ± 3.7 years and the mean follow up duration was 7.1 ± 3.2 years. Results: None of the 20 patients developed thalassemic facies, splenomegaly or hypersplenism. Sixteen patients maintained a normal weight (above 5th centile) and height (above 10thcentile), while 4 patients were at or below the 3rd centile. Of the 10 children above eleven years of age, 7 children reached normal puberty at their expected age. All 10 patients above eleven years of age, had a normal thyroid function test (TSH 2.27 ± 1.07 mIU/L, T4 11.04 ± 2.65 pmol/L) with no clinical or laboratory evidence of diabetes. Eight of the elder patients who had echocardiography had no evidence of pulmonary hypertension. Baseline bone densitometry done in 2 patients revealed a low Z-Score (below −2.5), with no improvement on follow-up. The mean total transfusion requirement was 162 ± 23 ml/kg/year. Patients were maintained on either deferiprone (n=14, dose 87 ± 12 mg/kg/day) or deferasirox chelation (n=6, dose 32 ± 4 mg/kg/day). Preliminary results of a self esteem questionnaire of these patients were better than our older patients who had not received hypertransfusion/chelation. Conclusion: TI children who were started at presentation on hypertansfusion/chelation regimen, have improved growth, better self esteem and they did not develop many of the complications known to occur in the non-transfused ones. However, this regimen needs to be evaluated in a larger prospective cohort study and to confirm the cost-effectiveness given the regular blood transfusion and continuous iron chelation. Disclosures: No relevant conflicts of interest to declare.


Author(s):  
Dr. Hitesh Kumar Solanki ◽  
Dr. Omnath P Yadav ◽  
Dr. Anita J Gojiya

The study was conducted in department of physiology, B J Medical College, Ahmedabad from Mar. 2012 to Feb. 2013. This was a cross-sectional study to evaluate the effect of smoking on lung   function and serum lipids in asymptomatic smokers   and comparable non   smokers. The mean of the various spirometric parameters were calculated of the subjects for both the groups. The mean FVC in group I and group II was 2.60 ± 0.62 L and 4.10 ± 0.64L respectively. The mean FEV1 in group I was 1.91 ± 0.57L and     3.19 ± 0.77L in group II Group I had mean FEF25% - 75% and PEFR of 1.98 ± 0.67L/sec and 4.50 ± 1.57L/sec respectively. Group II had mean FEF25 – 75% of 4.22 ± 1.23L/sec and a mean PEFR of 7.22 ± 1.42L/sec. In young smokers and asymptomatic, still the spirometric values were significantly deranged as compared to controls. Even smokers with history of less pack years of smoking also had significant abnormalities of lung function. All he spirometric values in the two groups had statistically highly significant difference and were higher in non-smokers as compared to smokers. The spirometric values were reduced in smokers with history of smoking for as low as two pack years. Keywords: Progression, PFT, Asymptomatic & Smokers


Author(s):  
Amrita S Kumar ◽  
A Geetha ◽  
Jim Joe ◽  
Arun Mathew Chacko

Introduction: Blood donation is one of the most significant contributions that a person can make towards the society. A donor generally donates maximum 450 mL of blood at the time of donation. If 450 mL of blood is taken in a donation, men lose 242±17 mg and women lose 217±11 mg of iron. Hence, adequate iron stores are very important in maintenance of the donor’s health. Aim: To assess the influence of frequency of blood donation on iron levels of blood donors by estimating Haemoglobin (Hb) and other blood indices which reflect iron status of blood and serum ferritin which reflects body iron stores. Materials and Methods: The present study was a cross-sectional analytical study, conducted on 150 blood donors, 18-40 years of age presenting to the Blood Bank in Government Medical College, Kottayam, Kerala, India, between December 2016 to December 2017. Total of 150 donors were divided into four groups according to the number of donations per year. Group I were the first time donors with no previous history of blood donation, Group II- included those with history of donation once in the previous year, Group III- those donors with history of donation twice in the previous year and Group IV- those having history of donation thrice in the previous year. Six ml of whole blood collected from each donor, two ml was used for estimating Haemoglobin (Hb), Packed Cell Volume (PCV), Mean Corpuscular Volume (MCV), Mean Corpuscular Hb (MCH), Mean Corpuscular Haemoglobin Concentration (MCHC) in haematology analyser. Serum separated from remaining four mL of blood underwent ferritin analysis by Chemiluminescence Immunoassay (CLIA) method. Iron stores were considered normal at serum ferritin value from 23.9-336ng/mL in males and 11-307ng/mL in females. Statistical analysis was performed in Statistical Package for the Social Sciences (SPSS) version 16.0. Analysis of Variance (ANOVA) test and Pearson correlation test were used to find association between various parameters and collected data. The p-value <0.05 was considered as statistically significant. Results: There was no significant correlation between serum ferritin level and frequency of blood donation. MCH, MCHC showed significant association (p-value 0.039 and 0.007, respectively) with frequency of blood donation. Low positive correlation was seen between Hb and PCV with serum ferritin levels (r=0.381, p-value <0.001 and r=0.354, p-value <0.001, respectively). Conclusion: There is no significant association between frequency of blood donation and serum ferritin levels.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 1631-1631
Author(s):  
Stefan Kuhle ◽  
Maria Spavour ◽  
Jacqueline Halton ◽  
Patricia Massicotte ◽  
Irene Cherrick ◽  
...  

Abstract BACKGROUND: Asymptomatic deep venous thrombosis (DVT) are well-known complications of treatment of acute lymphoblastic leukemia (ALL) in children. However, the clinical significance of radiographically detected, asymptomatic DVT is unclear and controversial, as there are no studies on long-term outcome of asymptomatic DVT in children available. There are two likely reasons for the studies not being done in this area. First, there is a lack of defined cohorts of pediatric patients screened for DVT and secondly, there is a great deal of difficulty in following patients over many years. The study, Prophylaxis with Antithrombin Replacement in Kids with ALL treated with L-Asparaginase (PARKAA) was a multicentre randomized controlled trial in which children with ALL were screened for DVT. As survivors of childhood cancer, the PARKAA cohort continues to be followed in their respective centers. Therefore, establishment of the PARKAA cohort (1997–99) and the ability to locate these patients provided a unique opportunity to study the long-term outcome of asymptomatic DVT. OBJECTIVE: To assess the incidence of PTS in children with ALL who previously had an asymptomatic DVT. The objective were approached in two ways. Firstly, to assess the outcome of asymptomatic DVT by determining the prevalence of PTS in children with a history of ALL with radiographically diagnosed DVT (PARKAA cohort); secondly, to corroborate the findings by determining the prevalence of PTS in an unselected group of survivors of childhood ALL. METHODS: Cross-sectional study in two separate populations: Group I comprised of children enrolled in the PARKAA multicentre study who had been screened for, and diagnosed with, DVT in the upper venous system. Group II consisted of non-selected patients &lt; 21 years with a history of ALL followed at Stollery Children’s Hospital, Edmonton. Patients were invited for a follow-up at their treatment centre (Group I) or were assessed for PTS childhood cancer survivor clinic (Group II). PTS was assessed by two of the investigators (Group I) or by the attending oncologist (Group II), respectively, using a standardized scoring sheet. RESULTS: Group I: 13 PARKAA patients with a history of ALL and objectively diagnosed DVT were assessed for PTS (4 males; median age 11.9 years; median age at diagnosis of ALL 4.4 years). 7/13 patients had PTS (54%, 95%CI 25;81). All patients with PTS had collaterals on examination, 3 also had increased arm circumference. Group II: 41 patients with a history of ALL were enrolled (61% males; median age at diagnosis 3.0 years; 28% high-risk, 67% standard risk). Mean length of follow-up since diagnosis was 9.5 years. PTS was diagnosed in 10/41 (24%; 95%CI 11–38) patients. All patients with PTS had collaterals on examination, 5 (50%) also had increased arm circumference. CONCLUSIONS: There is a clinically significant prevalence of PTS in children with a history of ALL and radiographically diagnosed DVT. A significant proportion of survivors of ALL develop PTS, indicating previously undiagnosed DVT in this population.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 3715-3715
Author(s):  
Ana Villegas ◽  
Ana Gaya ◽  
Emilio Ojeda ◽  
Ataulfo Gonzalez ◽  
Alvaro Urbano ◽  
...  

Abstract Background Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic, life threatening hematopoietic stem cell disorder with chronic hemolytic anemia, peripheral blood cytopenias and thrombosis Aims To observe the PNH clone and LDH evolution of the Spanish patients enrolled in the International PNH Registry, the thrombotic events and the role of eculizumab Methods We analyzed the 117 patients enrolled in the Registry until Dec. 31st 2012, classified in 3 groups: Classic/ hemolytic (group I, n 59), PNH with another bone marrow disorder (group II, n 42) and Subclinical (group III, n 14). The variables analyzed were PNH clone size, LDH levels, and incidence of thrombosis. Medians and percentages should be taken with caution due to the relatively small sample size. In addition to data collected in the Registry, additional patient information was obtained from local physicians. Results The median (range) age at presentation was 36.6 yrs. (16-83); 48 patients (41.0%) were women. Median (range) time from disease start to enrollment was 11.3 years in group I (0.1-41.2), 3.5 in II (0.1-33.8) and 3.4 (0.3-20.8) in III. A total of 49 patients (39 in group I) were started on eculizumab, 38 prior to enrollment (31 in group I) and 11 on or after enrollment; 3 were treated prior to enrollment but discontinued for different reasons (pregnancy, ending trial, access problems). Clone evolution (Table 1). In group I the median clone size remained stable during the follow-up period in the Registry; however, 4 patients in group II evolved to group I, with granulocyte clones > 50% and LDH levels >2000 U/L, while 3 initially in group I evolved to group II at 6, 12 and 18 months respectively. At enrollment 64 patients had a clone ≥30% and 31<30%; 7 patients in group II had a clone ≥30% despite hypoplasia, and they were treated with eculizumab. In groups I and II median clone size increased from Diagnosis to Enrollment in line with the physiopathology of the disease. LDH evolution (Table 2). Median LDH levels at diagnosis were higher in group I. In this group the decrease in LDH level between Diagnosis and Enrollment could be attributed to the start of treatment in 31 patients before the enrollment visit, but that hypothesis will need confirmation in future analysis. Thrombotic episodes (Table 3). Twenty six patients (22.6%) presented 52 TEs along the study period, 41 in group I and 11 in group II. Of the 26, fourteen presented 1 TE, six 2, one 3, four 2 and one 5. Twenty five patients presented 51 TEs since the moment of diagnosis while they were not being treated with eculizumab. Only one patient in the treated group presented a TE (CVA), of which he recovered well; after 30 months of the episode continues with the treatment and scores 90 in the Karnofsky index. Fifty eight percent of the patients presenting TEs were male, showing they may be more prone to TE than women. Conclusions These data show the dynamic features of the disease in some patients, which justifies the necessity of regularly monitoring the clone size LDH levels are higher in patients with classical PNH at diagnostic; the effect of the treatment in the whole cohort will require future analysis Thrombosis is highly prevalent in PNH; 22.6% of the patients in this sample had at least 1 episode along their time in the study. Disclosures: No relevant conflicts of interest to declare.


1997 ◽  
Vol 8 (8) ◽  
pp. 501-505 ◽  
Author(s):  
Monica Jonsson ◽  
Roger Karlsson ◽  
Ewa Rylander ◽  
Ake Gustavsson ◽  
Goran Wadell

The aim of this study was to determine the associations between risk behaviour and women's reported sexually transmitted diseases (STDs). All the women aged 19, 21, 23 and 25, residing in a specified housing area, were invited to answer a questionnaire regarding their sexual behaviour, smoking and alcohol consumption and previous history of STD. Of the 611 women participating, one out of 4 women had a history of at least one STD. In an univariate analysis, self-reported STD was found to be related to age, having more than 4 lifetime sexual partners, having practised intercourse at first date, inconsistent use of condoms, alcohol consumption of more than 3 bottles of wine per month and smoking. These factors were, however, not independent of each other and when subjected to a multivariate logistic regression analysis 2 factors, i.e. the lifetime number of sexual partners (more than 4 partners vs one; OR 7.94, (3.41-18.50)) and coitus on first date (practised more than once vs never, OR 2.99 (1.55-5.78)) emerged as independently associated with a previous STD.


Author(s):  
Chao Zhang ◽  
Leilei Zhang ◽  
Dongdong Wang ◽  
Haoli Ma ◽  
Bailin Liu ◽  
...  

Glycoside Hydrolase 3 (GH3) is a phytohormone-responsive family of genes that has been found in many plant species. It is implicated in the biological activity of indolacetic (IAA) and jasmonic acids (JA), and also affects plant growth and developmental processes and some stresses. In this study, GH3 genes were identified in 48 plants, which belong to algae, moss, fern, gymnosperm and angiosperm. No GH3 representative gene has been found in algae, and our research identified 4 genes in mosses, 19 in ferns, 7 in gymnosperms, and numerous in Angiosperms. The results showed that GH3 genes mainly occur in seed plants. Phylogenetic analysis of all GH3 genes showed three separate clades. Group I was related to JA adenylation, group II was related to IAA adenylation, and group III was separated from group II but the function was not clear. The structure of GH3 protein indicated highly conserved sequence in the plant kingdom. The analysis of JA-adenylation related to gene expression of GH3 in potato (Solanum tuberosum) showed that StGH3.12 highly responded to Methyl Jasmonate (MeJA) treatment. Expression levels of StGH3.1, StGH3.11, and StGH3.12 were high in flower and StGH3.11 expression was also high in stolon. Our research revealed the evolution of the GH3 family, which is useful for studying the precise function about JA-adenylation GH3 genes in S. tuberosum under development and biotic stresses.


2008 ◽  
Vol 45 (1) ◽  
pp. 11-16 ◽  
Author(s):  
Severino Marcos Borba de Arruda ◽  
Victorino Spinelli Toscano Barreto ◽  
Fernando José do Amaral

BACKGROUND: Presinusoidal portal hypertension with frequent episodes of upper gastrointestinal variceal bleeding are hallmarks of hepatosplenic Manson’s schistosomiasis; a clinical form that affects about 5% of Brazilians who are infected by Schistosoma mansoni. AIMS: To evaluate duplex sonography findings in patients with hepatosplenic Manson’s schistosomiasis with and without upper gastrointestinal variceal hemorrhage. METHODS: A cross-sectional study was performed whereby 27 consecutive patients with hepatosplenic Manson’s schistosomiasis were divided into two groups: group I (six men and six women; mean age 48.7 years) with a past history of bleeding and group II (four men and eight women; mean age 44.7 years) without a past history of upper gastrointestinal bleeding, underwent duplex sonography examination. All patients underwent the same upper gastrointestinal endoscopy and laboratory examinations. Those with signs of mixed chronic liver disease or portal vein thrombosis (three cases) were excluded. RESULTS: Group I showed significantly higher mean portal vein flow velocity than group II (26.36 cm/s vs 17.15 cm/sec). Although, as a whole it was not significant in all forms of collateral vessels (83% vs 100%), there was a significantly higher frequency of splenorenal collateral circulation type in group II compared with group I (17% vs 67%). The congestion index of the portal vein was significantly lower in group I than in group II (0.057 cm vs 0.073 cm/sec). CONCLUSION: Our duplex sonography findings in hepatosplenic Manson’s schistosomiasis support the idea that schistosomotic portal hypertension is strongly influenced by overflow status, and that collateral circulation seems to play an important role in hemodynamic behavior.


2017 ◽  
Vol 24 (01) ◽  
pp. 36-41
Author(s):  
Shafat Khatoon ◽  
Aijaz Ahmed ◽  
Nighat Jabeen ◽  
Erum Rehman

Cardiovascular diseases (CVDs) are the number one cause of death globally:more people die annually from CVDs than from any other cause. An estimated 17.5 millionpeople died from CVDs in 2012, representing 31% of all global deaths. Although CVDs areuncommon entity in young patients, it constitutes significant health problem due to itsdyslipidemia cases and devastating effects on active life style of young patients, it is thereforeimportant to identify diseases in young that are associated with or a cause of dyslipidemia1.Hypothyroidism is an important cause of dyslipidemia in young that can significantly increasethe risk of CVDs2. Objectives: This study is designed “to determine frequency of dyslipidemiain young hypothyroid patients”. Place and duration of Study: Study conducted at MedicalOPD JPMC, Karachi (outpatient) in six months duration from 25th May 2009 to 24th November2009. Patients and Methods: Study is performed on 100 newly diagnosed cases of primaryhypothyroidism between ages 25 to 55 years, non-smokers, having no previous history ofIschemic Heart Disease (IHD) or family history of premature CVD, diabetes mellitus (DM), hepaticor renal disease, not on drugs which could alter serum lipids. Selected case undergone 14hours fasting lipid profile check. Results: Out of 100 hypothyroid cases, 91% had dyslipidemiawhich was directly proportional to severity of hypothyroidism. Out of 100 hypothyroid cases,95 (95%) were of young age group i-e from 25-49 years, and all of them were dyslipidemic,while 05 (5%) hypothyroid patients were of age group more than 50 years and none of themhad dyslipidemia. (0.00%) and this distribution of dyslipidemic in young hypothyroid patientsis statistically significant (p value 0.031) Conclusion: Hypothyroidism is associated with highfrequency of dyslipidemia in young patients which significantly predisposes them to risks ofCVDs.


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