disorders of cerebrospinal and brain fluids chapter 48 hydrocephalus

... patients suffering from disorders and diseases of the brain, the advances in brain research provide hope in the form of their own understanding of what is going wrong and in the form of advances in novel ... Diseases and Disorders of the Human Brain, Edited by Theo Mantamadiotis p cm ISBN 978-953-51-0111-6 Contents Preface IX Part Chapter Chapter Syndromes and Disorders The Unique Properties of the ... symptoms of other disorders 30 When Things Go Wrong – Diseases and Disorders of the Human Brain 1.7 Externalizing and aggressive behavior in GTS The challenge of characterizing GTS per se is often...

Ngày tải lên: 28/06/2014, 11:20

... marrow Threefourths of the nucleated cells of bone marrow are committed to the production of leukocytes Leukocyte maturation in the marrow is under the regulatory control of a number of different factors, ... factors (CSFs) and interleukins (ILs) Because an alteration in the number and type of leukocytes is often associated with disease processes, total white blood count (WBC) (cells per µL) and differential ... final stages of maturation, no cell division occurs, and the cell passes through the metamyelocyte stage and then to the band neutrophil with a sausage-shaped nucleus (Fig 61-3) As the band cell...

Ngày tải lên: 07/07/2014, 01:20

... band form Döhle bodies are discrete, blue-staining nongranular areas found in the periphery of the cytoplasm of the neutrophil in infections and other toxic states They represent aggregates of ... characteristics and specific cell-surface markers are listed for each maturational stage Figure 61-3 Neutrophil band with Döhle body The neutrophil with a sausage-shaped nucleus in the center of the ... Stages of neutrophil development shown schematically G-CSF (granulocyte colony-stimulating factor) and GM-CSF (granulocytemacrophage colony-stimulating...

Ngày tải lên: 07/07/2014, 01:20

... only a subset of mature neutrophils The meaning of neutrophilheterogeneity is not known The morphology of eosinophils and basophils is shown in Fig 61-6 Figure 61-6 Normal eosinophil and basophil ... compartments: one freely flowing and one marginated The freely flowing pool is about one-half the neutrophils in the basal state and is composed of those cells that are in the blood and not in contact with ... CSFs, complement fragments, and chemokines, mobilize leukocytes from the bone marrow and deliver them to the blood in an unstimulated state Under normal conditions, ~90% of the neutrophil pool is...

Ngày tải lên: 07/07/2014, 01:20

... endothelial growth factor (VEGF), and prostaglandins E and I Cytokines regulate some of these processes [e.g., TNF-α induction of VEGF, interferon (IFN) γinhibition of prostaglandin E] In the healthy adult, ... been opsonized by IgG and C3b Fibronectin and the tetrapeptide tuftsin also facilitate phagocytosis With phagocytosis comes a burst of oxygen consumption and activation of the hexose-monophosphate ... oxidase, consisting of membrane and cytosolic components, is assembled and catalyzes the reduction of oxygen to superoxide anion, which is then converted to hydrogen peroxide and other toxic oxygen...

Ngày tải lên: 07/07/2014, 01:20

... for malignancy or control of autoimmune disorders These drugs cause neutropenia because they result in decreased production of rapidly growing progenitor (stem) cells of the marrow Certain antibiotics ... blood and bone marrow lymphocytosis, neutropenia, polyclonal hypergammaglobulinemia, splenomegaly, rheumatoid arthritis, and absence of lymphadenopathy Such patients may have a chronic and relatively ... associated with increased risk of infection than neutropenia of long duration (months to years) that reverses in response to infection or carefully controlled administration of endotoxin (see "Laboratory...

Ngày tải lên: 07/07/2014, 01:20

... to infection and other forms of acute inflammation and results from both release of the marginated pool and mobilization of marrow reserves Persistent neutrophilia with cell counts of ≥30,000–50,000/µL ... chronic inflammation and certain myeloproliferative diseases Increased marrow release and mobilization of the marginated leukocyte pool are induced by glucocorticoids Release of epinephrine, as ... and lower serum neutrophil-binding IgG Some Felty's syndrome patients also have neutropenia associated with an increased number of LGLs Splenomegaly with peripheral trapping and destruction of...

Ngày tải lên: 07/07/2014, 01:20

... features of the important inherited disorders of phagocyte function are shown in Table 61-4 Table 61-3 Types of Granulocyte and Monocyte Disorders Cause of Indicated Dysfunction ... Recessive) Severe infections of No respiratory NBT or DHR test; skin, ears, lungs, liver, and burst due to the lack of no superoxide and H2O2 bone with catalase-positive one of four NADPH production ... Recurrent infections of Abnormal skin, ears, and sinopulmonary chemotaxis, tract; delayed wound healing; respiratory decreased Lack of secondary impaired (specific) burst and neutrophils inflammation;...

Ngày tải lên: 07/07/2014, 01:20

... expression of leukocyte integrins on neutrophils or activated T and B cells Neutrophils (and monocytes) from patients with LAD adhere poorly to endothelial cells and protein-coated surfaces and exhibit ... leukocytosis (neutrophil counts of 15,000–20,000/µL) because cells not marginate; and, in severe cases, a history of delayed separation of the umbilical stump Infections, especially of the skin, may become ... slow healing, and development of dysplastic scars The most common bacteria are Staphylococcus aureus and enteric gram-negative bacteria LAD is caused by an abnormality of fucosylation of SLex (CD15s),...

Ngày tải lên: 07/07/2014, 01:20

... lymphocytes and neutrophils TNF-α is a pyrogen that duplicates many of the actions of IL-1 and plays an important role in the pathogenesis of gram-negative shock (Chap 265) TNF-α stimulates production of ... infected, they often have extensive inflammatory reactions, and lymph node suppuration is common despite the administration of appropriate antibiotics Aphthous ulcers and chronic inflammation of the ... Macrophages help regulate the replication of lymphocytes and participate in the killing of tumors, viruses, and certain bacteria (Mycobacterium tuberculosis and Listeria monocytogenes) Macrophages...

Ngày tải lên: 07/07/2014, 01:20

... leading to production of IL-2 and IFN-γ IFN-γ acts through its receptor on macrophages to upregulate TNF-α and IL-12 and kill intracellular parasites Mutant forms of the cytokines and receptors shown ... catalyzes the oxidation of many substances by hydrogen peroxide and may facilitate killing of microorganisms Eosinophil peroxidase, in the presence of hydrogen peroxide and halide, initiates mast ... cold urticaria, and Muckle-Wells syndrome Pyoderma gangrenosum, acne, and sterile pyogenic arthritis is caused by mutations in CD2BP1 In contrast to these syndromes of overexpression of proinflammatory...

Ngày tải lên: 07/07/2014, 01:20

... defenses is complex and poorly defined Laboratory Diagnosis and Management Initial studies of WBC and differential and often a bone marrow examination may be followed by assessment of bone marrow ... lymphocytes, macrophages, and fibroblasts accumulate in the affected tissues, but their role in pathogenesis is unclear Activation of eosinophils and fibroblasts and the deposition of eosinophilderived ... leukemia, and idiopathic hypereosinophilic syndrome (50,000–100,000/µL) The idiopathic hypereosinophilic syndrome represents a heterogeneous group of disorders with the common feature of prolonged...

Ngày tải lên: 07/07/2014, 01:20

... et al: Clinical features of dominant and recessive interferon-γ receptor deficiencies Lancet 364:2113, 2004 [PMID: 15589309] Etzioni A et al: Of man and mouse: Leukocyte and endothelial adhesion ... seen in patients with CGD on this regimen Oral quinolones such as levofloxacin and ciprofloxacin are alternatives In the setting of cytotoxic chemotherapy with severe, persistent neutropenia, trimethoprim-sulfamethoxazole ... patients, and patients with phagocytic cell dysfunction, should avoid heavy exposure to airborne soil, dust, or decaying matter (mulch, manure), which are often rich in Nocardia and the spores of Aspergillus...

Ngày tải lên: 07/07/2014, 01:20

... platelet activators The net effect of vasodilation and inhibition of platelet function is to promote blood fluidity, whereas the net effect of vasoconstriction and platelet activation is to promote ... the level of TPO, which then stimulates platelet production Platelets circulate with an average life span of 7–10 days Approximately one-third of the platelets reside in the spleen, and this number ... generation of intracellular signals that lead to activation of the platelet glycoprotein (Gp) IIb/IIIa (α IIbβ3) receptor and resultant platelet aggregation Activated platelets undergo release of their...

Ngày tải lên: 07/07/2014, 04:20

... tubes (purple top, often) used to collect blood for complete blood counts (CBCs) If a low platelet count is obtained in EDTA-anticoagulated blood, a blood smear can be evaluated and a platelet count ... (green-top tube), or ideally a smear of freshly obtained unanticoagulated blood, such as from a finger stick, can be examined Figure 109-1 Photomicrographs of peripheral blood smears A Normal ... platelet in autosomal dominant macrothrombocytopenia D Schistocytes microangiopathic hemolytic anemia and decreased platelets in ...

Ngày tải lên: 07/07/2014, 04:20

... risk of life-threatening hemorrhage in the thrombocytopenic patient Excessive bruising is seen in disorders of both platelet number and function Infection-Induced Thrombocytopenia Many viral and ... venous pressure, the ankles and feet in an ambulatory patient Petechiae are pinpoint, nonblanching hemorrhages and are usually a sign of a decreased platelet number and not platelet dysfunction ... production and platelet survival In addition, immune mechanisms can be at work, as in infectious mononucleosis and early HIV infection Late in HIV infection, pancytopenia and decreased and dysplastic...

Ngày tải lên: 07/07/2014, 04:20

... thrombocytopenia, timing of platelet count drop, thrombosis and other sequelae such as localized skin reactions, and other cause of thrombocytopenia not evident Figure 109-3 Time course of heparin-induced ... associated with bleeding and, in fact, markedly increases the risk of thrombosis HIT results from antibody formation to a complex of the plateletspecific protein platelet factor (PF4) and heparin The ... surface antigens and result in thrombocytopenia only when the drug is present Many drugs are capable of inducing these antibodies, but for some reason they are more common with quinine and sulfonamides...

Ngày tải lên: 07/07/2014, 04:20

... in treatment of HIT, with prompt discontinuation of heparin and use of alternative anticoagulants Thrombosis is a common complication of HIT, even after heparin discontinuation, and can occur ... Introduction of warfarin alone in the setting of HIT or HITT may precipitate thrombosis, particularly venous gangrene, presumably due to clotting activation and severely reduced levels of proteins C and ... secondary causes of ITP and should include testing for HIV infection and hepatitis C (and other infections if indicated); serologic testing for SLE; serum protein electrophoresis and immunoglobulin...

Ngày tải lên: 07/07/2014, 04:20

... anomaly and Sebastian, Epstein's, and Fechtner syndromes, all of which have distinct distinguishing features A common feature of these disorders is large platelets (Fig 109-1C) Autosomal recessive disorders ... microangiopathies are a group of disorders characterized by thrombocytopenia, a microangiopathic hemolytic anemia evident by fragmented RBCs (Fig 109-1D) and laboratory evidence of hemolysis, and microvascular ... thrombocytopenia and microangiopathy are seen, a coagulopathy predominates, with consumption of clotting factors and fibrinogen resulting in an elevated prothrombin time (PT) and often activated...

Ngày tải lên: 07/07/2014, 04:20

... so clear, and fear of withholding treatment, as well as lack of other treatment alternatives, results in broad application of plasma exchange However, withdrawal, or reduction in dose, of endothelial ... cyclophosphamide, and splenectomy The role of rituximab in the treatment of this disorder needs to be defined A significant relapse rate is noted: 25–45% within 30 days of initial "remission" and 12–40% ... The pathogenesis of inherited (Upshaw-Schulman syndrome) and idiopathic TTP is related to a deficiency of, or antibodies to, a metalloprotease that cleaves vWF and ADAMTS13, respectively...

Ngày tải lên: 07/07/2014, 04:20