ENA and MAKOTO 1 Case:Jessica 3on3 Publishing Series (Japanese Edition)
Selective bias intrinsic in testing for AAT deficiency and the rarity of the Pi SS genotype will make future study of this association dependent on population-based tests. Full Text Available Background. Full Text Available Abstract Background Infertility is a major problem in sickle cell disease patients, especially in males. In addition to low serum testosterone, other abnormalities involving the accessory sex organs, such as the seminal vesicles and the prostate gland, as well as marked decrease in ejaculate volume may be observed in male Hb SS patients.
Hence, the need to study the role of sex hormones as a cause of infertility in male Hb SS patients. Methods An unmatched case-control study was performed using seventy-five consenting subjects from Lagos University Teaching Hospital. These included 47 patients with haemoglobin phenotype SS from the Sickle cell clinic and 28 volunteered medical students and members of staff with haemoglobin phenotype AA. Demographic data were obtained using a self-administered questionnaire. A total of 5 mls of blood was collected from each subject between 9. Results The concentrations of serum testosterone in Hb SS patients ranged from 0.
Determination of glycated hemoglobin in patients with advanced liver disease. To evaluate the glycated hemoglobin HbA 1c determination methods and to determine fructosamine in patients with chronic hepatitis, compensated cirrhosis and in patients with chronic hepatitis treated with ribavirin. HbA1c values were determined in 15 patients with compensated liver cirrhosis and in 20 patients with chronic hepatitis using the ion-exchange high performance liquid chromatography and the immunoassay methods.
Fructosamine was determined using nitroblue tetrazolium. Forty percent of patients with liver cirrhosis had HbA1c results below the non-diabetic reference range by at least one HbA1c method, while fructosamine results were either within the reference range or elevated. Twenty percent of patients with chronic hepatitis hepatic fibrosis had HbA1c results below the non -diabetic reference range by at least one HbA1c method. Only evaluated in context with all liver function parameters as well as a red blood count including reticulocytes, HbA 1c results should be used in patients with advanced liver disease.
HbA 1c and fructosamine measurements should be used with caution when evaluating long-term glucose control in patients with hepatic cirrhosis or in patients with chronic hepatitis and ribavirin treatment. Background Fetal hemoglobin level is a heritable complex trait that strongly correlates swith the clinical severity of sickle cell disease. Only few genetic loci have been identified as robustly associated with fetal hemoglobin in patients with sickle cell disease , primarily adults.
The sole approved pharmacologic therapy for this disease is hydroxyurea, with effects largely attributable to induction of fetal hemoglobin. Studies assessing influences of specific sequence variants in these and other genetic loci in larger populations and in unusual hydroxyurea responders are needed to further understand the maintenance and therapeutic induction of fetal hemoglobin in pediatric sickle cell disease. Many severely affected HbSC patients have been placed on hydroxyurea on a case by case basis, but there are no large scale prospective data on safety or efficacy of hydroxyurea in this subset of patients with SCD.
Here, we report a case series of 14 pediatric patients with HbSC treated to maximum tolerated dose MTD with hydroxyurea. Those who failed to show clinical improvement after at least six months at MTD were offered phlebotomy in addition to hydroxyurea. Of the four placed on dual hydroxyurea and phlebotomy therapy, all showed at least partial clinical improvement. A marked rise in percent dense cells preceded clinical complications in three patients.
Dual therapy with hydroxyurea and phlebotomy may be an effective approach to patients with HbSC that do not experience improvement with hydroxyurea alone. Large scale clinical trials are needed to evaluate the safety and efficacy of hydroxyurea and hydroxyurea with phlebotomy in patients with HbSC disease. Characterization of systemic disease in primary Sjogren's syndrome: To reach a European consensus on the definition and characterization of the main organspecific extraglandular manifestations in primary SS.
The purpose of our study was to determine the synergistic protective effects of mitochondria-targeted antioxidant SS 31 and mitochondria division inhibitor 1 Mdivi1 in Alzheimer's disease AD. Using biochemical methods, we also measured cell survival and apoptotic cell death. Ours is the first study to investigate combined treatment of mitochondria-targeted antioxidant SS 31 and mitochondrial division inhibitor 1 in AD neurons. The value of measuring levels of glycated hemoglobin HbA1c for the prediction of first cardiovascular events is uncertain.
To determine whether adding information on HbA1c values to conventional cardiovascular risk factors is associated with improvement in prediction of c Hemoglobin to Hematocrit Ratio: Femoral head osteonecrosis ON secondary to sickle cell disease SCD often progresses to femoral head collapse, requiring total hip arthroplasty. However, this treatment has a limited durability and patients with SCD have higher rates of complications, requiring multiple revision operations.
Identifying risk factors linked to ON in SCD can facilitate earlier precollapse diagnosis and surgical treatment aimed at preservation of the native hip joint. Two sided t-tests assuming unequal variances determined statistically risk factors and threshold values were assigned to calculate odds ratios. There was no observed correlation between ON and height, body mass index, cholesterol, mean corpuscular volume, hematocrit, or glucocorticoid use. High-risk children should receive hip magnetic resonance imaging to diagnose early ON and facilitate interventions focused on hip preservation, forestalling, or possibly preventing.
Acquired hemoglobin H disease in a patient with aplastic anemia evolving into acute myeloid leukemia. The prognosis of severe aplastic anemia has improved since the introduction of bone marrow transplantation and treatment with antithymocyte globulin. In contrast to the success of these protocols, studies with long term follow-up have shown the occurrence of clonal diseases such as paroxysmal nocturnal hemoglobinuria, myelodysplastic syndrome and acute leukemia in aplastic anemia. We report the first case of a Brazilian patient with aplastic anemia who developed myelodysplastic syndrome and acute myeloid leukemia showing acquired hemoglobin H and increased fetal hemoglobin.
Hemoglobin is the major protein found in erythrocytes, where it acts as an oxygen carrier molecule. In recent years, its expression has been reported also in neurons and glial cells, although its role in brain tissue remains still unknown. Altered hemoglobin expression has been associated with various neurodegenerative disorders. In addition, expression analysis of the specific erythrocyte marker ALAS2 was performed in order to account for blood contamination of the tissue samples. In addition, while in sporadic and genetic forms of prion disease hemoglobin transcripts did not shown any alterations, both chains display a strong down-regulation in AD brains.
These results were confirmed also at a protein level. These data indicate distinct hemoglobin transcriptional responses depending on the specific alterations occurring in different neurodegenerative diseases. In particular, the initial site of misfolding event central nervous system vs. We describe the findings from a national study of maternal and fetal outcomes of pregnancy in women with sickle cell disease SCD. Data included antenatal, maternal and fetal outcomes.
There was no difference in the incidence of acute chest syndrome, hypertension and venous thromboembolism between Hb SS and HbSC. Women with Hb SS were more likely to deliver at pregnancy. Full Text Available Background: The KIz SS network, a sentinel surveillance system for infectious diseases in day care centers: Full Text Available Abstract Background Day care-associated infectious diseases are widely recognized as a public health problem but rarely studied.
Insights into their dynamics and their association with the day care setting are important for effective decision making in management of infectious disease control. This paper describes the purpose, design and potential of our national multi-center, day care-based sentinel surveillance network for infectious diseases the KIz SS network. The aim of the KIz SS network is to acquire a long-term insight into the syndromic and microbiological aspects of day care-related infectious diseases and associated disease burden and to model these aspects with day care setting characteristics.
Data on infectious disease symptoms and related morbidity children and staff, medical consumption, absenteeism and circulating enteric pathogens children are collected on a daily, weekly or monthly basis. Every two years, a survey is performed to assess the characteristics of participating day care centers. Discussion The KIz SS network offers a unique potential to study infectious disease dynamics in the day care setting over a sustained period of time.
The created biodatabases will help us to assess day care-related disease burden of infectious diseases among attending children and staff and their relation with the day care setting.
This will support the much needed development of evidence-based and pragmatic guidelines for infectious disease control in day care centers. Hemoglobin , hematocrit, and changes in cerebral blood flow: Hemoglobin and hematocrit are important determinants of blood viscosity and arterial oxygen content and may therefore influence cerebral blood flow CBF.
We examined cross-sectional and prospective associations of hemoglobin and hematocrit with CBF in patients with manifest arterial disease. Hemoglobin H Hb H disease , however, has been rarely reported. We decided therefore toverify the rarity of disease and characterize the presenting features incases identified. All patients seen in Qatif Central Hospital betweenSeptember and November with low red cell indices were screened forHb H disease and those found positive had clinical data compiled from theirhospital records and analyzed.
Thirty-nine cases of Hb H were diagnosed. Themean age of patients was 18 years. The mean hemoglobin was The mean Hb Bart's level was In addition to low redcell indices, all patients had a high red cell distribution width RDW meanof The main clinical signs were jaundice and hepatosplenomegaly. Concurrent glucosephosphate Dehydrogenase G6PD deficiency was seen in The red cell indices may mimic iron deficiency, whichshould be excluded by the presence of jaundice and organomegaly.
Thecondition often co-exists with G6PD deficiency. Abnormal hemoglobin levels are a risk factor for Alzheimer's disease AD. Although the mechanism underlying these associations is elusive, inadequate micronutrients, particularly folate and vitamin B12, may increase the risk for anemia, cognitive impairment, and AD. In this study, we investigated whether the nutritional status of folate and vitamin B12 is involved in the association between low hemoglobin levels and the risk of AD mortality. We categorized three groups based on the quartiles of folate and vitamin B12 as follows: Of 4, participants, 49 subjects died due to AD.
After adjusting for age, sex, ethnicity, education, smoking history, body mass index, the presence of diabetes or hypertension, and dietary intake of iron, significant increases in the AD mortality were observed in Quartile1 for hemoglobin HR: This association did not exist in subjects with at least one high level of folate and vitamin B Our finding shows the relationship between folate and vitamin B12 levels with respect to the association between hemoglobin levels and AD mortality.
Full Text Available Chronic kidney disease CKD patients, characterized by traditional and nontraditional risk factors, are prone to develop atheromatosis and thus cardiovascular events and mortality. The angiogenesis of the adventitial vasa vasorum aVV surrounding the carotid has been described as the atheromatosis initiator. Therefore, the aim of the study was to 1 evaluate if the carotid aVV in CKD patients increases in comparison to its physiological value of healthy patients; 2 explore which traditional or nontraditional risk factor including inflammation, bone and mineral metabolism, and anemia could be related to the aVV angiogenesis.
CKD patients without previous cardiovascular events 44, stages ; 37, stage 5D and 65 healthy subjects were compared. CKD patients at stages showed higher aVV of the right carotid artery even after adjusting for age. In conclusion, the association of hemoglobin with higher aVV could suggest the role of high hemoglobin in the higher incidence of adverse cardiovascular outcomes in CKD patients. Splenic infarction at low altitude in a child with hemoglobin S-C disease. We describe a year-old black boy with hemoglobin S-C disease living in Atlanta altitude 1, ft , with no prior history of aircraft or mountain travel, who developed splenic infarction.
The clinical picture was characterized by severe left upper quadrant abdominal pain, fever, splenomegaly, and hematologic and scintigraphic evidence of functional asplenia. Treatment consisted of analgesics, intravenous fluids, and short-term antibiotic therapy. The child recovered without sequelae. Full Text Available Globalization causes high mobility of human and livestock, hence increase the transmission of infectious diseases , including avian influenza, severe acute respiratory syndrome SARS, and swine influenza.
Therefore, prevention of those diseases is required. Vaccines are effective to prevent infectious diseases ; however, their development takes a long time and they cannot provide immediate protection in pandemic cases. The primary mechanism of these technologies is inhibition of gene expression, typically by causing the destruction of specific RNA molecule of the pathogen. The use of gene silencing technologies is expected to give new alternative that is more effective in eradication of infectious diseases in animals before threaten human being.
Incidence of sickle cell disease and other hemoglobin variants in 10, Lebanese neonates. Full Text Available Hemoglobinopathies are highly prevalent diseases and impose a public health burden. Early diagnosis and treatment can ameliorate the course of these diseases and improve survival.
Despite purported high incidence of hemoglobinopathies in Lebanon, there are no nationwide screening programs. In this study, newborn screening utilizing high pressure liquid chromatography was executed in all public hospitals across Lebanon between and All newborns with an abnormal hemoglobin Hb were offered genetic counseling and all those with disease were enrolled in comprehensive hemoglobinopathy clinics.
The majority of those with SCD had non-Lebanese origins. The most common causes of hospitalizations in infants with SCD were acute splenic sequestration and pain crises. No bacteremia or other life threatening infections were noted. At a median follow up 14 months follow up range 7 to 34 months, all children with disease are alive and compliant with treatment.
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Systematic screening for SCD and other Hb variants was shown to be feasible, cost effective, and of accurate predictive value. This program was also clinically effective because it led to the identification of babies with disease and to providing them with free early multidisciplinary care. Conclusively, a newborn screening program should be implemented across Lebanon to detect hemoglobinopathies and initiate early therapeutic and preventive strategies and genetic counseling. Hemoglobin and hematocrit levels in the prediction of complicated Crohn's disease behavior--a cohort study.
Markers that predict the occurrence of a complicated disease behavior in patients with Crohn's disease CD can permit a more aggressive therapeutic regimen for patients at risk. The aim of this cohort study was to test the blood levels of hemoglobin Hgb and hematocrit Hct for the prediction of complicated CD behavior and CD related surgery in an adult patient population. The relation of these markers and clinical events was studied using Kaplan-Meier survival analysis and adjusted COX-proportional hazard regression models. The median follow-up time was Low Hgb or Hct levels were independent predictors of a shorter time to occurrence of the first complication or CD related surgery.
This was true for early as well as late occurring complications. Stable low Hgb or Hct during serial follow-up measurements had a higher frequency of complications compared to patients with a stable normal Hgb or Hct, respectively. The effect of hematocrit and hemoglobin on the risk of ischemic heart disease: A Mendelian randomization study. Hematocrit and hemoglobin affect viscosity, and have been considered as risk factors for ischemic heart disease IHD , although observations are inconsistent; randomized controlled trials targeting hematocrit or hemoglobin have not been definitive.
To clarify their role, the risk of IHD was assessed according to genetically determined hematocrit and hemoglobin. We applied single nucleotide polymorphisms SNPs strongly determining hematocrit and hemoglobin , from a genome wide association study, to a large case 64, control , study of coronary artery disease , CARDIoGRAMplusC4D, to obtain unconfounded estimates using instrumental variable analysis by combining the Wald estimators for each SNP taking into account any correlation between SNPs using weighted generalized linear regression.
In conclusion, hematocrit shares genetic determinants with IHD, but whether the genes contribute to IHD via hematocrit or other mechanisms is not entirely clear. Higher Hemoglobin is unlikely to cause IHD. Linear regression analyses showed that greater hemoglobin and hematocrit values were associated with lower baseline parenchymal CBF and more decline in parenchymal CBF over time, independent of cardiovascular risk factors, use of antiplatelet drugs, anticoagulants, or diuretics, and brain measures: Higher hemoglobin and hematocrit were associated with lower baseline parenchymal CBF and a greater decline in parenchymal CBF over time, possibly as a result of physiological compensating mechanisms.
Sonographic markers including cardiothoracic CT ratio and middle cerebral artery peak systolic velocity MCA-PSV were serially assessed and recorded from the first trimester. This may help us identify Hb Bart's disease among fetuses at risk in earlier gestation and proper schedules for serial ultrasound could be made more effectively. Current hemoglobin levels are more predictive of disease progression than hemoglobin measured at baseline in patients receiving antiretroviral treatment for HIV type 1 infection.
Hemoglobin is the most important component of red blood cells. It is composed of a protein called Abnormalities of an individual's hemoglobin value can indicate defects in the normal balance Hemoglobin and atherosclerosis in patients with manifest arterial disease.
Decreased hemoglobin levels are known to be associated with an increased risk of coronary mortality and morbidity. This is largely thought to result from the development of left ventricular hypertrophy. Similar remodeling mechanisms of the vessel wall that may result in atherosclerosis are likely to. Peripheral vascular response to mild indirect cooling in patients with homozygous sickle cell SS disease and the frequency of painful crisis. In homozygous sickle cell SS disease , skin cooling is a common precipitating factor of the painful crisis which is associated with avascular necrosis of active bone marrow.
Since skin cooling does not directly induce sickling, we have investigated the nature of the reflex vascular responses to mild cooling in SS patients in a steady state of the disease and compared them with their history of painful crises. Experiments were performed in Jamaica on 60 male SS patients and 30 matched control subjects with normal haemoglobin AA genotype. Cutaneous erythrocyte flux in forearm and hand was monitored by a laser Doppler meter.
The contralateral hand was immersed in cool water at 16 degrees C for 2 min, 6 times, at random intervals of 0. However, the direction of change in FVR varied between individuals such that 18 AA subjects showed an increase in FVR constrictor group while 12 showed a decrease in FVR, indicating vasodilatation in forearm muscle dilator group. Significance of measurement of serum somatostatin SS , growth hormone GH , insulin-like growth factor-1 IGF-1 and some related cytokines levels in patients with graves' disease. To study the possible roles of the related hormones and cytokines in the pathogenesis of Graves' disease.
Clinical, hematological and genetic data of a cohort of children with hemoglobin SD. The hemoglobin FSD is very uncommon in newborn screening programs for sickle cell disease. In the program of Minas Gerais, Brazil, the clinical course of children with hemoglobin SD was observed to be heterogeneous. The objective of this study was to estimate the incidence and to describe the natural history of a cohort of newborns with hemoglobin SD. Isoelectric focusing was the primary method used in newborn screening. Polymerase chain reaction-restriction fragment length polymorphism and gene sequencing were used to identify mutant alleles and for haplotyping.
Gap-polymerase chain reaction was used to detect alpha-thalassemia. Other variants with hemoglobin D mobility were not identified. Transcranial Doppler was normal in all children. In contrast, children with hemoglobin S-Korle Bu had clinical course and blood cell counts like children with the sickle. Wilson's disease WD is a copper deposition disorder which can result in a number of extrapyramidal motoric symptoms such as parkinsonism. Therefore, this study was carried out to investigate, for the first time, nigrostriatal dopaminergic function in WD in relation to different courses and severity of the disease.
Using high-resolution single-photon emission tomography SPET after administration of 2 ss -carbomethoxy-3 ss - 4[ I]iodophenyl tropane [ I] ss -CIT , striatal dopamine transporters DAT were imaged in 43 WD patients and a control group of ten subjects. The SPET data were compared with clinical data on the course of the disease CD , the severity of neurological symptoms and the degree of hepatic alteration. For the different subgroups a tendency was detected towards a stepwise decrease in the specific [ I] ss -CIT binding ratios from pseudo-sclerosis CD 9.
Vertebrate hemoglobin , contained in erythrocytes, is a globular protein with a quaternary structure composed of 4 globin chains 2 alpha and 2 beta and a prosthetic group named heme bound to each one. Having myoglobin as an ancestor, hemoglobin acquired the capacity to respond to chemical stimuli that modulate its function according to tissue requirements for oxygen.
Fish are generally submitted to spatial and temporal O2 variations and have developed anatomical, physiological and biochemica Primary biliary cirrhosis PBC may be associated with various rheumatological disorders. To investigate the frequency and significance of 'rheumatological' antinuclear antibodies in the field of autoimmune chronic liver disease , with special regard to PBC.
We studied patients with PBC, autoimmune liver disease controls type 1 and 2 autoimmune hepatitis, primary sclerosing cholangitis , 30 systemic lupus erythematosus and 50 blood donors. Sera were tested for the presence of antibodies to extractable nuclear antigens anti-ENA by counterimmunoelectrophoresis, enzyme-linked and immunoblot IB assay, and for the presence of anti-centromere antibodies ACA by indirect immunofluorescence on HEp-2 cells and IB.
Effect of scaling and root planing on erythrocyte count, hemoglobin and hematocrit in patients with chronic periodontal disease. Anemia of chronic disease , a cytokine-mediated anemia, is a frequent complication of many chronic inflammatory conditions. The present clinical trial was aimed to evaluate the effect of chronic periodontal disease on erythrocyte count, hemoglobin and hematocrit and the changes produced in these parameters after the provision of periodontal therapy.
Group A was categorized as chronic generalized gingivitis, and Group B was categorized as chronic generalized periodontitis on the basis of clinical findings. Complete oral prophylaxis was performed for all patients. Patients were recalled after 3 weeks and 3 months. The clinical and hematological parameters were re-evaluated to analyze the changes after provision of phase I therapy. Lower values of EC, Hb and HCT in Group B showed that mild anemia is associated with chronic generalized periodontitis, which tends to improve after provision of periodontal therapy.
Minimal changes in MCV, MCH and MCHC indicated that the lower values are not due to any vitamin and mineral deficiencies, but secondary to the chronic inflammatory changes associated with chronic periodontal disease.
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Genetic heterogeneity of hemoglobin AEBart's disease: Genotypic and phenotypic features are heterogeneous. We studied the hematologic and molecular characteristics of this disease in a cohort of Thai patients encountered at our center in northeast Thailand. It was found that all these forms of AEBart's disease showed similar thalassemia intermedia phenotypes but those with non-deletional forms were relatively more anemic. Our data confirm that in such area with high prevalence of hemoglobinopathies such as Southeast Asia, identification of rare thalassemia alleles in a thalassemia intermedia patient should not be ignored.
Careful consideration of different phenotypic expression may help in providing presumptive diagnosis of this disease where access to molecular testing is limited. However, molecular diagnostic is useful for predicting the clinical outcome and improving genetic counseling of these complex hemoglobinopathies. Full Text Available In neuronal systems, the health and activity of mitochondria and synapses are tightly coupled.
Therefore, the prevention or rescue of mitochondrial dysfunction may help delay or altogether prevent AD-associated neurodegeneration. Since mitochondrial health is heavily dependent on antioxidant defenses, researchers have begun to explore the use of mitochondria-targeted antioxidants as therapeutic tools to prevent neurodegenerative diseases.
This review will highlight advances made using a model mitochondria-targeted antioxidant peptide, SS 31, as a potential treatment for AD. Association of adiposity with hemoglobin levels in patients with chronic kidney disease not on dialysis. The article Association of adiposity with hemoglobin levels in patients with chronic kidney disease not on dialysis, written by Hirokazu Honda, Kota Ono, Tadao Akizawa, Kosaku Nitta and Akira Hishida, was originally published electronically on the publisher's internet portal currently springerlink on November 4, without open access. The original article was corrected.
Full Text Available Vertebrate hemoglobin , contained in erythrocytes, is a globular protein with a quaternary structure composed of 4 globin chains 2 alpha and 2 beta and a prosthetic group named heme bound to each one. Fish are generally submitted to spatial and temporal O2 variations and have developed anatomical, physiological and biochemical strategies to adapt to the changing environmental gas availability.
Structurally, most fish hemoglobins are tetrameric; however, those from some species such as lamprey and hagfish dissociate, being monomeric when oxygenated and oligomeric when deoxygenated. Fish blood frequently possesses several hemoglobins ; the primary origin of this finding lies in the polymorphism that occurs in the globin loci, an aspect that may occasionally confer advantages to its carriers or even be a harmless evolutionary remnant. On the other hand, the functional properties exhibit different behaviors, ranging from a total absence of responses to allosteric regulation to drastic ones, such as the Root effect.
Low fetal hemoglobin percentage is associated with silent brain lesions in adults with homozygous sickle cell disease. Silent white matter changes WMCs on brain imaging are common in individuals with sickle cell disease SCD and are associated with cognitive deficits in children. We investigated the factors predictive of WMCs in adults with homozygous SCD and no history of neurological conditions. Patients were recruited from a cohort of adults with homozygous SCD followed up at an adult sickle cell referral center for which steady-state measurements of biological parameters and magnetic resonance imaging scans of the brain were available.
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A systematic review of known mechanisms of hydroxyurea-induced fetal hemoglobin for treatment of sickle cell disease. To report on molecular mechanisms of fetal hemoglobin HbF induction by hydroxyurea HU for the treatment of sickle cell disease. Studies have provided consistent associations between genomic variations in HbF-promoting loci and variable HbF level in response to HU. Three main molecular pathways have been reported: Research on post-transcriptional mechanisms could lead to therapeutic targets that may minimize alterations to the cellular transcriptome.
Rheological Variations among Nigerians with Different Hemoglobin However, previous studies have not addressed whether the ability of lentiviral vectors to increase hemoglobin synthesis might vary in different patients. Among the thalassemic patients, we identified a subset of specimens in which hemoglobin production can be achieved using fewer copies of the vector integrated than in others.
Our results suggest two major findings. This approach would provide vital information to select the best candidates for these. We report three rare cases: High tryptophan diet reduces CA1 intraneuronal ss -amyloid in the triple transgenic mouse model of Alzheimer's disease.
FH - Neurology Impact factor: Hb F levels are higher 0. Can glycated hemoglobin act as a reliable glycemic indicator in patients with diabetic chronic kidney disease? Chronic kidney diseases CKD is a common microvascular complication in patients with diabetes mellitus DM which requires adequate glycemic control. Glycated hemoglobin HbA1c is a conventional biomarker to estimate glycemic status, but its role in diabetic CKD patients is unclear. Therefore, this study aimed to determine whether patients with high HbA1c are associated to develop diabetic CKD.
Data were obtained from a clinical registry of diabetic patients who were tre The impact of the treatment method on intradialytic intercurrences and serum levels of hemoglobin , calcium and albumin of the person with chronic kidney disease. The high prevalence of chronic kidney disease has led to the development of renal function replacement techniques as a treatment for individuals with the disease. Hemodialysis HD is the most commonly used method for the treatment of patients with the disease. Even if it has improved a lot, the intercurrences during dialysis continue to be a reality.
The literature is not consensual when it relates the modalities of treatment and the benefits and harms associated with them. If on the one hand there is evidence that hemodiafiltration HDF has fewer implications for patients than hemodialysis, others do not show significant differences when comparing the two treatment modalities. A prospective, comparative, quasi-experimental study was developed over a period of 8 months to determine if there were differences in the number of intercurrences and serum levels of calcium, albumin and hemoglobin observed in each treatment modality, HD versus HDF.
The data obtained show that there are no statistically significant differences in the number of intercurrences and serum levels of calcium, albumin and hemoglobin observed in each treatment modality. Biochemical Properties and Clinical Correlates. Diseases affecting hemoglobin synthesis and function are extremely common worldwide. These variants alter hemoglobin structure and biochemical properties with physiological effects ranging from insignificant to severe.
Studies of these mutations in patients and in the laboratory have produced a wealth of information on hemoglobin biochemistry and biology with significant implications for hematology practice. More generally, landmark studies of hemoglobin performed over the past 60 years have established important paradigms for the disciplines of structural biology, genetics, biochemistry, and medicine. Here we review the major classes of hemoglobin variants, emphasizing general concepts and illustrative examples.
Sickle cell disease is a growing global health concern because infants born with the disorder in developing countries are now surviving longer with little access to diagnostic and management options. To inform future screening efforts in Haiti, we assayed sickle hemoglobin mutations using traditional hemoglobin solubility tests HST and add-on techniques, which incorporated spectrophotometry and insoluble hemoglobin separation. We also generated genotype data as a metric for HST performance. We found 19 of individuals screened with HST were positive for sickle hemoglobin , five of whom did not carry the HbS allele.
We show that spectrophotometry and insoluble hemoglobin separation add-on techniques could resolve false positives associated with the traditional HST approach, with some limitations. We also discuss the incorporation of insoluble hemoglobin separation observation with HST in suboptimal screening settings like Haiti. On the dopplars SS A new family of X-ray sources has recently been proposed including SS Particularly relevant for the verification of the model are the observations at the phase of minimum shift.
Acute non-atherosclerotic ST-segment elevation myocardial infarction in an adolescent with concurrent hemoglobin H-Constant Spring disease and polycythemia vera. Full Text Available Thrombosis is a major complication of polycythemia vera PV and also a well-known complication of thalassemia.
Coronary artery angiography revealed an acute clot in the right coronary artery without atherosclerotic plaque. He was treated with plateletpheresis, hydroxyurea and antiplatelet agents. The platelet count decreased and his symptoms improved. This case represents the importance of early diagnosis, awareness of the increased risk for thrombotic complications, and early treatment of PV in patients who have underlying thalassemia with marked thrombocytosis. There are no strategies to prevent SR and treatments are extremely limited.
The present study evaluated 1 the retinal pigment epithelial RPE cell as a hemoglobin producer and novel cellular target for fetal hemoglobin HbF induction, and 2 monomethylfumarate MMF as an HbF-inducing therapy and abrogator of oxidative stress and inflammation in SCD retina. The production of hemoglobin by RPE suggests the potential involvement of this cell type in the etiology of SR. Monomethylfumarate influences multiple parameters consistent with improved retinal health in SCD and may therefore be of therapeutic potential in SR treatment. The Hemoglobin E Thalassemias. Hemoglobin E HbE is an extremely common structural hemoglobin variant that occurs at high frequencies throughout many Asian countries.
Alpha chain hemoglobins with electrophoretic mobility similar to that of hemoglobin S in a newborn screening program. To characterize alpha-chain variant hemoglobins with electric mobility similar to that of hemoglobin S in a newborn screening program. Clinical and hematological data were obtained from children who attended scheduled medical visits.
The following alpha chain variants were found: Two associations with hemoglobin S were found: There was no evidence of clinically relevant hemoglobins detected in this study. The hemoglobins detected in this study may lead to false diagnosis of sickle cell trait or sickle cell disease when only isoelectric focusing is used in neonatal screening. Additional tests are necessary for the correct identification of hemoglobin variants. Pharmacist-led, primary care-based disease management improves hemoglobin A1c in high-risk patients with diabetes.
We developed and evaluated a comprehensive pharmacist-led, primary care-based diabetes disease management program for patients with Type 2 diabetes and poor glucose control at our academic general internal medicine practice. The primary goal of this program was to improve glucose control, as measured by hemoglobin A1c HbA1c. Clinic-based pharmacists offered support to patients with diabetes through direct teaching about diabetes, frequent phone follow-up, medication algorithms, and use of a database that tracked patient outcomes and actively identified opportunities to improve care.
Baseline HbA1c averaged In predictive regression modeling, baseline HbA1c and new onset diabetes were associated with significant improvements in HbA1c. Age, race, gender, educational level, and provider status were not significant predictors of improvement. In conclusion, a pharmacist-based diabetes care program integrated into primary care practice significantly reduced HbA1c among patients with diabetes and poor glucose control. Phylogeny of Echinoderm Hemoglobins.
In contrast, very little is known about hemoglobins in echinoderms, a phylum of exclusively marine organisms closely related to vertebrates, beyond the presence of coelomic hemoglobins in sea cucumbers and brittle stars. We identified about 50 hemoglobins in sea urchin, starfish and sea cucumber genomes and transcriptomes, and used Bayesian inference to carry out a molecular phylogenetic analysis of their relationship to vertebrate sequences, specifically, to assess the hypothesis that the neuroglobin and cytoglobin lineages are also present in echinoderms.
The genome of the sea urchin Strongylocentrotus purpuratus encodes several hemoglobins , including a unique chimeric domain globin, 2 androglobin isoforms and a unique single androglobin domain protein. Other strongylocentrotid genomes appear to have similar repertoires of globin genes. We carried out molecular phylogenetic analyses of 52 hemoglobins identified in sea urchin, brittle star and sea cucumber genomes and transcriptomes, using different multiple sequence alignment methods coupled with Bayesian and maximum likelihood approaches. The results demonstrate that there are two major globin lineages in echinoderms, which are related to the vertebrate neuroglobin and cytoglobin lineages.
Furthermore, the brittle star and sea cucumber coelomic hemoglobins appear to have evolved independently from the cytoglobin lineage, similar to the evolution of erythroid oxygen binding globins in cyclostomes and vertebrates. The presence of echinoderm globins related to the vertebrate neuroglobin and cytoglobin lineages suggests that the split between neuroglobins and cytoglobins occurred in the deuterostome ancestor shared by echinoderms and vertebrates.
The ratio of hematocrit Hct to hemoglobin Hb in the people with normal red blood cell RBC morphology is generally three to one. The Hct and Hb levels were derived by automated analyzer. A ratio of 3.
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Relativistic jets in SS The most unusual characteristic of the star SS emerged in the late 's when a series of optical spectra showed intense, broad optical emission lines whose profiles and wavelengths changed drastically from night to night. These features are interpreted as strong Doppler-shifted Balmer and HeI lines.
The modulation of the Doppler shifts are observed as being cyclic with a period of about days. It was hypothesized that these phenomena were caused by two collimated, colinear, jets which were ejecting in opposite directions from SS Most authors believe that velocity variations of the emission lines are caused by a cyclic rotation of jet axis inclined to line of sight. This rotation being the result of precession, which leads one to suspect SS as a member of a close binary system.
This hypothesis has been confirmed from recent optical, radio, and x-ray observations which are discussed in the article. The combination of optical and radio observations of SS , described in the article, gives an accurate measure of the Kinematics of the system and some confidence that the Kinematic equations are understood. However, the specific physical processes of this ejection are poorly understood. Some theoretical difficulties regarding this are given.
Data were obtained from a clinical registry of diabetic patients who were treated in a district hospital in the Northeast of Thailand. Blood samples are widely used for PCR-based DNA analysis in fields such as diagnosis of infectious diseases , cancer diagnostics, and forensic genetics.
Also, electrophoretic mobility shift assay was applied to investigate interactions between inhibitory proteins and DNA, and isothermal titration calorimetry was used to directly measure effects on DNA polymerase activity. Whole blood caused a decrease in the number of positive digital PCR reactions, lowered amplification efficiency, and caused severe quenching of the fluorescence of the passive reference dye 6-carboxy-X-rhodamine as well as the double-stranded DNA binding dye EvaGreen.
Immunoglobulin G was found to bind to single-stranded genomic DNA, leading to increased quantification cycle values. Hemoglobin affected the DNA polymerase activity and thus lowered the amplification efficiency. Hemoglobin and hematin were shown to be the molecules in blood responsible for the fluorescence quenching. In conclusion, hemoglobin and immunoglobulin G are the two major PCR inhibitors in blood, where the first affects amplification through a direct effect on the DNA polymerase activity and quenches the fluorescence of free dye molecules, and the latter binds to single-stranded genomic DNA, hindering DNA polymerization in the first few PCR cycles.
Hemoglobins Hbs corresponding to non-symbiotic nsHb and truncated tHb Hbs have been identified in rice Oryza. This review discusses the major findings from the current studies on rice Hbs. At the molecular level, a family of the nshb genes, consisting of hb1, hb2, hb3, hb4 and hb5, and a sin Approximately , Americans have sickle cell disease SCD , a group of recessively inherited red blood cell disorders characterized by abnormal hemoglobin , called hemoglobin S or sickle hemoglobin , in the red blood cells.
Red blood cells that contain sickle hemoglobin are inflexible and can stick to vessel walls, causing a blockage that slows or stops blood flow. When this happens, oxygen cannot reach nearby tissues, leading to attacks of sudden, severe pain, called pain crises, which are the clinical hallmark of SCD. The red cell sickling and poor oxygen delivery can also cause damage to the brain, spleen, eyes, lungs, liver, and multiple other organs and organ systems.
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These chronic complications can lead to increased morbidity, early mortality, or both. Tremendous strides in treating and preventing the complications of SCD have extended life expectancy. Association of grade of non-alcoholic fatty liver disease and glycated albumin to glycated hemoglobin ratio in patients with type 2 diabetes mellitus. This retrospective, cross-sectional study was performed with data obtained from T2DM patients. NAFLD was defined as ultrasonographically detected fatty liver and was graded as normal, mild, moderate, and severe fatty liver.
Profile of glycated- hemoglobin , antioxidant vitamin and cytokine levels in pulmonary tuberculosis patients: Uncontrolled blood glucose, which marked by high level of HbA1c, increases risk of pulmonary TB because of cellular immunity dysfunction. This study aimed to analyze profile of glycated hemoglobin , antioxidant vitamins status and cytokines levels in active pulmonary TB patients. Study subject consisted of 62 pulmonary TB patients, diagnosed with positive acid fast bacilli and chest X-ray. Mean HbA1c was 7. This study supports the importance of performing diabetes screening among pulmonary TB patients.
Further study needs to be done to determine the feasibility of TB-DM co-management. Bizarre spectrum of SS SS is an emission-line star, a radio source, and an x-ray source centered in a supernova remnant, W Through observations and spectroscopic studies much has been learned of this object. The spectrum of SS is characterized by Doppler-shifted emission lines of hydrogen and helium. This implies the presence of a gas.
The wavelengths of these lines vary. Some of the moving lines of the spectrum are red-shifted and some are blue-shifted. Since the wavelengths of the Doppler-shifted features change rapidly with time, the velocity of both approaching gas and receding gas is changing. Also, the variations in velocity are periodic, with a day period. These characteristics of the spectrum of SS are explained by assuming the following hypothetical model. The object responsible for ejecting two jets in opposite directions is thought to be part of a binary system, consisting of a comparatively normal star bound in close orbit to a neutron star, which is in the process of pulling material away from the companion by virtue of its strong gravitational field.
The gas streaming from the normal star forms a rotating accretion disk around the neutron star, and it is from the faces of this disk that the two jets are ejected in opposite directions. Precession of the plane of the disk is presumably what causes the axis of the jets to rotate, hence the day period. Oxygen transport is altered in hemoglobinopathies. To study the distribution of hemoglobinopathies in Andean subjects without African ancestry. The frequency and type of hemoglobinopathy was established by capillary and agarose gel electrophoresis.
The frequency of hemoglobinopathies was The structural variants found were: Quantitative variants found were Hb A-Beta thalassemia All techniques showed good correlation and capillary electrophoresis demonstrated a greater detection of hemoglobin variants. The frequency of hemoglobin variants in the analyzed population was high, which is an important public health indicator. Capillary electrophoresis can discern any Hb variants present in the population. Both low and high hemoglobin levels lead to more physical diseases , and both are linked to mortality.
Low hemoglobin , often classified as anemia, has also been linked to more depressive symptoms, but whether both hemoglobin extremes are associated with depressive disorder and potentially. Streptococcus suis serotype 2 S. Due to the failure of the current inactivated vaccine to protect against the disease , development of a new attenuated live vaccine against S. Our previous results have shown that Ss Pep plays a critical role in the pathogenesis of S.
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Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells Adults with sickle cell anemia Hb SS are inconsistently treated with hydroxyurea. We retrospectively evaluated the effects of elevating fetal hemoglobin with hydroxyurea on organ damage and survival in patients enrolled in our screening study between and An electronic medical record facilitated development of a database for comparison of study parameters based on hydroxyurea exposure and dose. This study is registered with ClinicalTrials.
Three hundred eighty-three adults with homozygous sickle cell disease were analyzed with 59 deaths during study follow-up. Hydroxyurea use was not associated with changes in organ function over time. While alkaline phosphatase was lowest in patients with the best fetal hemoglobin response Our data suggest that adults should be treated with the maximum tolerated hydroxyurea dose, ideally before organ damage occurs. Prospective studies are indicated to validate these findings. Sickle cell disease is one of the commonest and most studied genetic diseases in the world.
Abnormal Hb S molecules suffer polymerization physiologically provoked by a low oxygen tension, acidosis and dehydration. As a result, red blood cells take on a sickle cell form, which causes microvascular occlusion with varying consequences. The objective of this study was to review the importance of fetal hemoglobin in the clinical assessment of sickle cell.
Utility of Capillary Electrophoresis. A very tiny peak of Hb CS of the mother was easily ignored on the high performance liquid chromatography chromatogram while it was clearly seen on the capillary electrophoresis CE electrophoregram. This is of potential benefit for prevention of new cases of Hb H-CS disease. Management efficiency improvement promotion of SS ; SS no unei koritsuka sokushin. Full amount fund petroleum product marketer and Sumisyo petroleum of Sumitomo accelerate management efficiency improvement of service station SS.
National about places have been developed in within the year Within SS , it aims at break-even point achievement of gasoline, coarse advantage 10 yen per light oil of 1 liter in 84 all tied SS stores. But, by doing personnel configurations and operational procedures, that they reexamine the balance management, etc. Technology has transformed the practice of medicine and surgery in particular over the last several decades. This change in practice has allowed diagnostic and therapeutic tests to be performed less invasively.
Hemoglobin monitoring remains one of the most commonly performed diagnostic tests in the United States. Recently, non-invasive hemoglobin monitoring technology has gained popularity. The aim of this article is to review the principles of how this technology works, pros and cons, and the implications of non-invasive hemoglobin technology particularly in trauma surgery.
Published by Elsevier Ltd. Nonlinear photoacoustic spectroscopy of hemoglobin. As light intensity increases in photoacoustic imaging, the saturation of optical absorption and the temperature dependence of the thermal expansion coefficient result in a measurable nonlinear dependence of the photoacoustic PA signal on the excitation pulse fluence. Here, under controlled conditions, we investigate the intensity-dependent photoacoustic signals from oxygenated and deoxygenated hemoglobin at varied optical wavelengths and molecular concentrations.
The wavelength and concentration dependencies of the nonlinear PA spectrum are found to be significantly greater in oxygenated hemoglobin than in deoxygenated hemoglobin. These effects are further influenced by the hemoglobin concentration. These nonlinear phenomena provide insights into applications of photoacoustics, such as measurements of average inter-molecular distances on a nm scale or with a tuned selection of wavelengths, a more accurate quantitative PA tomography.
Louis, One Brookings Drive, St. Louis, Missouri United States. Advanced drug delivery nanosystems aDDn Ss: Significant progress has been made in nanoscale drugs and delivery systems employing diverse chemical formulations to facilitate the rate of drug delivery and to improve its pharmacokinetics. Biocompatible nanomaterials have been used as biological markers, contrast agents for imaging, healthcare products, pharmaceuticals, drug-delivery systems as well as in detection, diagnosis and treatment of various types of diseases.
The classification of drug delivery nanosystems DDn Ss is a crucial issue and fundamental efforts on this subject are missing from the literature. The classification was based on their characteristics such as: The aDDn Ss can be classified as hybridic Hy- or chimeric Chi- based on the nature - same or different respectively - of biomaterials and inorganic materials used. Amazon Giveaway allows you to run promotional giveaways in order to create buzz, reward your audience, and attract new followers and customers. Learn more about Amazon Giveaway. Jessica 3on3 Publishing Series Japanese Edition.
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