hemolytic anemias red cell membrane and metabolic defects

... the ghost cells was measured which was the same at t ¼ and h This indicates that the 14 C sucrose was trapped by loading and resealing and did not leak out of the ghost cells The ghost cells were ... inside red cell ghosts upon the rubidium uptake of those modifiers Error bars represent standard deviation Fig SDS/PAGE of red cell ghosts with and without a-crystallin resealed Lanes and 5, molecular ... of haemoglobin were prepared by gel filtration chromatography [18] Blood (5–6 mL) was centrifuged (1000 g) for 10 min, the plasma and white cells aspirated and the red cells resuspended at °C with...

Ngày tải lên: 17/03/2014, 03:20

... Anemia and Age / 28 Anemia and Ethnicity / 38 Anemia and Pregnancy / 45 Cancer and Anemia / 58 Malaria and the Red Cell / 81 SECTION II: NUTRITION AND ANEMIA Iron Deficiency / 97 95 Nutritional Anemias ... of red cells to determine red cell mass A second and more commonly used technique is centrifugation of a blood sample, which packs the red cells by centripetal force and leaves the plasma and ... of red cells is a reasonable practice as long as the number of red cells is in the normal range Anemias by definition have fewer red cells than normal Consequently, the use of a percentage of red...

Ngày tải lên: 10/08/2014, 08:20

... phase of red cell invasion and multiplication Red cells are constantly created and then destroyed as they age and senesce A mutation that somehow destroys both the infected red cells and the parasite ... the serum Both iron deficiency and chronic hemolytic anemias increase the test readout Red cell ZPP ZPP content of red cells Iron-deficient erythropoiesis increases red cell ZPP values Conditions ... trait red cells P falciparum parasites cultured in sickle trait red cells die when the cells are incubated at low oxygen tension.15 In contrast, parasite health and growth were unimpeded in cells...

Ngày tải lên: 10/08/2014, 08:20

... alters the CBC Impaired erythrocyte production dramatically lowers the total red cell count, hemoglobin, and hematocrit The large size CHAPTER NUTRITIONAL ANEMIAS 137 of the red cells translates ... THF Adomet methionine synthase reductase methionine synthase THF methylcobalamin Extracellular Intracellular homocysteine Cell Membrane FIGURE 8–4 Cellular uptake and metabolism of cobalamin Cobalamin ... anemia The red cells are large and have an oval shape The most striking feature is the neutrophil with seven nuclear lobes The nucleated red cell has two irregular nuclei while other cells have...

Ngày tải lên: 10/08/2014, 08:20

... excess of 10,000 cells/μL reduce the risk of catastrophic bleeds, such as intracranial hemorrhage ᭿ PURE RED CELL APLASIA Pure red cell aplasia reflects selective destruction of red cell progenitors ... of red cell membrane CD59 The 1937 demonstration by Thomas H Ham that hemolysis of PNH red cells occurs via the action of complement on abnormal red cells22 reflected CD59 deficiency in these cells ... androgen therapy include masculinization, severe acne, and hepatic damage (hepatic peliosis and liver tumors) Supportive treatment with red cell and platelet transfusions then is often required...

Ngày tải lên: 10/08/2014, 08:20

... nuclear genome When cells undergo mitosis, the organelles distribute randomly to the daughter cells Acquired mitochondrial defects therefore pass unevenly to the daughter cells.130 This property ... links between the function of nuclear genes and the mitochondrion ACQUIRED SIDEROBLASTIC ANEMIAS Acquired sideroblastic anemias substantially exceed hereditary forms in frequency The disorder sometimes ... Coproporphyrinogen oxidase activity and porphyrin concentrations in peripheral red blood cells in hereditary sideroblastic anaemia Scand J Haematol 34:235–237 238 STEM CELL DYSFUNCTION 81 82 83 84...

Ngày tải lên: 10/08/2014, 08:20

... Heinz bodies also adhere to the red cell membrane where they disrupt architecture, promote oxidation and cross-linking of lipids and proteins, and impair function of membrane- associated enzymes including ... of membrane structures produce rigid red cells with impaired ability to pass through capillaries CHAPTER 15 G6PD DEFICIENCY 313 FIGURE 15–2 Heinz body prep of G6PD deficient cells The red cells ... the cytoskeleton to the membrane by forming a bridge between β spectrin and the intrinsic membrane protein, band Actin and protein 4.2 are also prominent members of the red cell cytoskeleton (From...

Ngày tải lên: 10/08/2014, 08:20

... infection, 188–189 red cell distribution width (RDW) value, 11 red cell fragmentation syndrome, 59 red cell membrane cytoskeleton, 332, 332f refractory anemia, 211 cytopenia, peripheral blood cell line ... or red cell sensitization.10 The drug induces red cell autoantibodies in 10–20% of patients after or more months of use These red cell antibodies are true autoantibodies directed against red ... expressed on the red cell membrane Glycoproteins are quite immunogenic and most provoke some degree of antibody production when introduced into a foreign environment Most of the red cell membrane glycoproteins...

Ngày tải lên: 10/08/2014, 08:20

... immediately after milking and stored on ice Milk cells were harvested by centrifugation at 3200 g for 20 at °C, and the cellular fraction was washed in NaCl/Pi buffer and processed for total RNA ... threonine and proline as the predominant amino acids, however, their high contents of leucine and asparagine is a characteristic shared only with the MUC8, MUC9, MUC13, and MUC16 Like the membrane- associated ... MUC15 in human and bovine tissues and cell types MUC15 mRNA expression was examined by PCR screening of commercial multiple tissue cDNA panels and oligo(dT) primed cDNA libraries and by RT-PCR...

Ngày tải lên: 24/03/2014, 04:21

... Determinant of Red Cell Shape 34 Membrane Proteins 35 Separation and Identification of Membrane Proteins 35 Membrane Proteins and Membrane Structure 36 Membrane Proteins in the Red Cell Surface 37 Membrane ... appeared to be related to possible 1.3 The Dawn of Red Cell Membrane Research abnormalities in the red cell membrane constituents It is well known that red cell membranes are composed mainly of membrane ... red cells), lipids (5.1 mg mLÀ1 of the red cells), and carbohydrates (80 mg mLÀ1 of the red cells) The red cell membranes are composed of 19.5 % (w/w) water, 39.6 % proteins, 35.1 % lipids, and...

Ngày tải lên: 29/06/2014, 11:20

... quite different, and they will be considered separately Hemolytic Anemias Anemias due to increased destruction of red cells, or hemolytic anemias (HAs), may be inherited or acquired From the clinical ... of Hemolytic Anemiasa Intracorpuscular Defects Hereditary Hemoglobinopathies Extracorpuscular Factors Familial hemolytic uremic syndrome (HUS) Enzymopathies Membrane- cytoskeletal defects Acquired ... correlation between hereditary causes and intracorpuscular defects, because such defects are due to inherited mutations; the one exception is PNH, because the defect is due to an acquired somatic mutation...

Ngày tải lên: 07/07/2014, 04:20

... damage to the membrane or to failure of the cation pump In either case the life span of the red cell is reduced, which is the definition of a hemolytic disorder If the rate of red cell destruction ... remaining 120 days of the red cell "life" span As a result of this unique process of differentiation and maturation, intermediary metabolism is drastically curtailed in mature red cells (Fig 101-1); ... decreases as red cells age Another consequence of the relative simplicity of red cells is that they have a very limited range of ways to manifest distress under hardship: in essence, any sort of metabolic...

Ngày tải lên: 07/07/2014, 04:20

... all HAs is an increased red cell turnover The gold standard for proving that the life span of red cells is reduced (compared to the normal value of about 120 days) is a red cell survival study, ... sometimes referred to as aplastic crisis Inherited Hemolytic Anemias There are three essential components in the red cell: (1) hemoglobin, (2) the membrane- cytoskeleton complex, and (3) the metabolic ... feature and hypersplenism may develop, with consequent neutropenia and/ or thrombocytopenia The increased red cell turnover also has metabolic consequences In normal subjects, the iron from effete red...

Ngày tải lên: 07/07/2014, 04:20

... a dimer of α-spectrin and β-spectrin The membrane is physically linked to the cytoskeleton by a third set of proteins (including ankyrin and the so-called band 4.1 and band 4.2), which thus connect ... almost invariably inherited mutations, and thus diseases of the membrane- cytoskeleton complex belong to the category of inherited hemolytic anemias Before the red cells lyse, they often exhibit more ... century as hereditary spherocytosis (HS) and hereditary elliptocytosis (HE) Their molecular basis has been elucidated Hereditary Spherocytosis This is a relatively common type of hemolytic anemia,...

Ngày tải lên: 07/07/2014, 04:20

... Very rare; s antigen nonspherocytic associated with hemolytic total loss of all anemia Rh antigens Note: HS, hereditary spherocytosis; HE, hereditary elliptocytosis ... Band 4.1 HE (dominant) Mutations of this gene account for about 5% of mostly HE, with prominent morphology but no hemolysis in heterozygotes; severe hemolysis in homozygotes EPB42 15q15-q21 Band ... some severe forms ANK1 8p11.2 Ankyr in HS (dominant) May account for majority of HS SLC4A 17q21 Band HS (anion (dominant) channel) Mutations of this gene may account for ~25% of HS Southea st Polymorp...

Ngày tải lên: 07/07/2014, 04:20

... one hand, as in many other HAs, the spleen itself is a major site of destruction; on the other hand, transit through the splenic circulation makes the defective red cells more spherocytic and ... germ cell of one of his parents or early after zygote formation; and (2) the patient may have a recessive form of HS (Table 101-3) In most cases the diagnosis is confirmed on the basis of red cell ... 101-3) and from the clinical point of view Again it is the shape of the red cells that gives the name to these conditions, but there is no direct correlation between elliptocytic morphology and...

Ngày tải lên: 07/07/2014, 04:20

... Table 101-4 Red Cell Enzyme Abnormalities Causing Hemolysis Enzyme (Acronym) Chro Pre Cli mosomal valence of nical Location Enzyme Commen ts Manifest Deficiency ations (Rank) ExtraRed Cell Gly Hexokina ... transfusions; sometimes it is mild, bordering on a nearly compensated hemolytic disorder As a result, the diagnosis may be delayed, and in some cases it is made in young adults—for instance, in a woman ... 3q11– Rar e (3) May benefit from (P5N) splenectomy Note: CNS, central nervous system; AHA, acquired hemolytic anemia ...

Ngày tải lên: 07/07/2014, 04:20

... in most tissues, this is less marked than in red cells, and it does not seem to produce symptoms Figure 101-4 Diagram of redox metabolism in the red cell G6P, glucose 6-phosphate; 6PG, 6-phosphogluconate; ... Abnormalities of Redox Metabolism G6PD Deficiency Glucose 6-phosphate dehydrogenase (G6PD) is a housekeeping enzyme critical in the redox metabolism of all aerobic cells (Fig 101-4) In red cells, its ... because it is the only source of reduced nicotinamide adenine dinucleotide phosphate (NADPH), which, directly and via reduced glutathione (GSH), defends these cells against oxidative stress G6PD...

Ngày tải lên: 07/07/2014, 04:20

... polychromasia, and spherocytes (Fig 101-6) The most typical feature is the presence of bizarre poikilocytes with red cells that appear to have unevenly distributed hemoglobin (hemighosts) and red cells ... precipitates of denatured hemoglobin and regarded as a signature of oxidative damage to red cells (except for the rare occurrence of an unstable hemoglobin) LDH is high and so is the unconjugated ... Mediterranean Sea, in the Middle East, and in India; G6PD A– in Africa and in Southern Europe; G6PD Vianchan and G6PD Mahidol in Southeast Asia; G6PD Canton in China; and G6PD Union worldwide The heterogeneity...

Ngày tải lên: 07/07/2014, 04:20

... activation red through any red cell population pathway cells Paroxysmal Acute Immune Test for Often cold lysis of normal Donath- triggered hemoglobinuria red cells viral infection (PCH) Landsteiner ... Other organisms may Clostridium be responsible perfringens Microangio pathic Acute or chronic Red cell Red cell Different fragmentation morphology on causes ranging blood smear from endothelial damage ... older assay d by ingestion fraction of large dish of of G6PDdeficient fava beans; but red trigger can be cells infection drug instead or ...

Ngày tải lên: 07/07/2014, 04:20