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43 Cards in this Set
- Front
- Back
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sickle cell disease
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precipitation of Hbg subject to hypoxia; erythrocytes fragile and rigid; adhere to endothelium and may pile up in capillaries; mutation in gene encoding B-globin chain of hemoglobin A
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hereditary spherocytosis
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autosomal dominant; rbcs sphere-shaped and lack central pallor; hemolysis; mutation in ankyrin most common but also spectrin, band 3, band 3; membrane transport activities affected; osmotically fragile; splenomegaly
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leukemoid reaction
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persistent neutrophilia b/c redistribution of neutrophils; total wbc over 50K; redist from marrow, microcirculation; caused by inflam, infection, malignancy; LAP is>100
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chronic myelogenous leukemia/chronic granulocytic leukemia
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proliferating cells partly/completely differentiated; myeloctyes, metamyelocytes, bands, neutrophils in peripheral blood; total wbc >100K; increased EOS/BAS; LAP<10; splenomegaly
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Charcot Leyden crystals
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phospholipase B crystals in diseases associated with eosinophilia; seen in secretions/excretions/CT; sputum of asthmatics
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cellularity of the bone marrow
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hypercellular marrow bc tumors of hematopoietic cell (acute myelogenous leukemia); calculated by subtracting an individual's age from 100 and addine +/- 10%; a 67 yr old would have 23-43% of active bone-producing cells; hypocellular bc of aplastic anemia or chemotherapy
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thrombocytopenia (TCP)
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platelet count <150,000/ul; cause of decreased platelet production is alcohol; impairs maturation of megakaryocytes; rise after 3-5 days of abstinence
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chronic granulomatous disease (CGD)
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X-linked; deficient NADPH oxidase in neutrophils and monocytes; respiratory burst(RB) is absent; nitroblue tetrazolium test (NBT) where neutrophils change to blue is RB is intact; test involving oxidation of dihydrorhodamine to fluorescent rhodamine; HOCl not made and bacteria not killed; in CT bc neutrophils replaced by cells associated with chronic inflammation (lymphocytes/macrophages); macrophages fuse to make multinucleated giant cells
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monocytosis
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greater than 10% of total wbc; greater than 800/ul; hodgkin lymphoma and chronic myelogenous leukemia cause it
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conditions that cause eosinopneia and relations to endocrine system
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1) dosage with glucocorticoids 2) dosage with ACTH 3) stressers cause relase of adrenal cortisol (burn, trauma) 4)cushing disease, H in cushing causes hypercortisolism, hypertension, hyperglycemia, hirsutism
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megaloblastic anemia
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reduced rbc, low hemoglobin, low rbc, low hematocrit; impaired DNA syn bc vit B12 or folic acid deficiency; nuclear development impaired but cytoplasm normal; large, nucleated erythroblasts called megaloblasts (basophilic/muddy cytoplasm); destruction in bone marrow; releases macrocytic or macrocytes
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Acquired Immunodeficiency Disease (AIDs)
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mediated by RNA retrovirus called Human Immunodeficiency Virus (HIV); uses glycoprotein (gp120) on surface to bind to CD4 protein on T helper cells; reverse transcriptase converts RNA to DNA and incoporated into genome of T cell; replicates and lyzes T cells to release more HIV; results in decrease CD4+ T cells; immune system responds to infections by generating CD8+ CTL and Ab; diagnose with PCR/CD4+ counts; treat with AZT (inhibits reverse transcriptase)
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Lymphedema
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abnormal swelling of arm/leg; 1)primary=inherited, improper development of lymphatic vessels (a)Milroy-infancy, malformation of lymph nodes (b)Meige-childhood, lack of valves, backward flow; 2)secondary=from surgery, radiation treatment, cancerous mass, infection
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Lymphangiomas
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benign tumors; due to incomplete development; failure to connect with venous system, obstruction of flow, form cysts, impair breathing; treat with OK-432 (Grp A strep+penicillin, stimulates inflamm response, neutrophils, macrophages, NK cells, endothelium permeable and lymph drains)
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Cystic hygromas
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accumulation of fluid around neck and head; 5% stillbirths; associated with chromosomal abnormalities; swelling of lymph, decreases intravascular vol, cardiac failure
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Lymphatic filariasis
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nematode worms, bites from mosquitos; larveal worms enter and circulate in bloodstream at night; adults in lymph vessels and cause blockage; elephantiasis; kill larvae, treat for 6 yeras (albendazole, mectizan/heartgard, diethylcarbamazine)
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Tonsilitis
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tonsil can impair swallowing/breathing; Group A streptococcal bacteria; antibiotics; reoccuring abscess forms causing hypertrophic tonsils
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Enlarged Lymph Nodes
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infection; migration/activation of B cells to nodules in cortex of lymph nodes; proliferate rapidly; may be hard/palpable; diagnostic for upper respiratory infection
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Autoimmune disease
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no longer can distinguish self from non-self; lymphocytes see Ags from self as foreign and destroy healthy tissue; ex:RA, graves disease, lupus; immunosuppressive drugs
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DiGeorge syndrome
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absense of thymus and parathyroid glands from abnormal dev of 3rd/4th pharyngeal pouches; normal humoral but abnormal cell-mediated immunity; die from tetany (no parathyroid) or infection (no thymus)
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Hodgkin's Lymphoma
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malignant growth; Reed-Sternberg cells (B-cell derived); 40/50% associated with epstein-barr virus; enlarged lymph nodes, petechia due to decrease in platelets, weight loss, fatigue; age 15-40 or >55; spleno/heptamegaly
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Diabetes insipidus
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abnormal production of dilute urine (20L) and excessive thirst; decreased ADH bc hypothalamus/post pituitary lesion; increases permeability of nephron
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Pituitary adenomas
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tumor of anterior pituitary gland; enlarge or supress secretions by pars distalis; destroy bone and nerual tissues
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Thyroid hormones and fetal development
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T3/T4 cross placental barrier; important for early stage of brain development; also stimulate gene expression for GH; congenital hypothyroidism combination of CSN damage and stunt growth
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Simple goiter
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enlargement of thyroid gland because insufficient iodine and excessive TSH from anterior pituitary; could be autoimmune (Hashimoto's Thyroiditis)- Ab against thyroglobulin, thyroid peroxidase/TSH receptor, apoptosis of thyroid follicular cells; low T3/T4 cause increased secretion of TSH
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Graves disease
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autoimmune; binding of anti-TSH Abs to TSH receptors on thyroid follicular cells; enlarge thryoid and protrusion of eyeballs; increased hormones and decreased follicular colloid; increase metabolism and sympathetic activity
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Hyperparathyroidism
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overactive parathyroid glands result in excess PTH and bone resorption; high blood Ca levels, deposition of Ca salts in kidneys (stones) and bvs; caused by benign tumor
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Addison disease
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secretion of inadequate mineralocorticoid and glucocorticoids (adrenocorticol hormones); due to destruction of adrenal cortex; autoimmune or tuberculosis; hyperpigmentation; steroid treatment
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Pinealoma with Parinaud syndrome
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dorsal midbrain syndrome; tumor of pineal gland affecting eye movement and pupil function; disrupt melatonin production causing insomnia; upward gaze from compression at superior colliculus and CN 3; disruption of hypothalamic inhibitory pathways (beta-hCG secretion and Leydig cell stimulation and testosterone)
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Diabetes mellitus
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Type 1) insulin-dependent, juvennile-onset, low insulin, damange to beta cells; Type 2) noninsulin-dependent, not from low insulin, insulin-resistant because decreased binding of insulin to its receptor, defects in induction of signal transduction pthwy
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Multiple endocrine neoplasmia
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2 or more tumors of endocrine glands; MEN1-associated w/pituitary adenoma, parathyroid hyperplasias, pancreatic tumors; MEN2- parathyroid hyperplasias, medullary pheochromocytoma, thyroid carcinoma; Lincoln had MEN type 2B, large bumpy lips
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Zollinger-Ellison syndrome
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gastrin-secreting tumors of pancreatic islets or sm intestine; hyperplasia/ hypertrophy of fundis; high acid secretion independent of food ingestion; H+ secretion continues bc pancreatic gastrin cannot be regulated by neg feedback
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Pemphigus vulgaris
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chronic, blistering skin disorder caused by IgG Ab on surface Ag (desmoglein, plakin) on keratinocytes; autoimmune
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Epidermolysis bullosa
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intraepidermal blister; basal cells look like tombstones; Type 2 hypersensitivity rxn; 3 mechanisms of blister formation: defects in keratin within basal layer, defect in laminin, defect in type 7 collagen-anchoring fibrils; diseases of the epidermal-dermal interface
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Sweating and disease-cystic fibrosis-sweat test
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sweat composition can be an indicator of disease; in uremia (kidneys) urea is in sweat
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Skin repair
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primary after surgical incision; secondary when separated edges and more loss of cells and tissues, generating granulation tissue; blood clot, platelets release factors, neutrophils/macrophages, KGFs, MMPs by Ks, MMPs cut Type4/7 collagen releasing Ks from BL, MMPs also cut Type 1/2 to aid mvmt, gran tissue, reepithelialization, prolif of fibroflasts, deposition of ECM
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Scleroderma
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excessive collagen in dermis; sklerosis can occur in other organs; tightening of the skin; radial furrowing around lips; Raynaud's phenomena; Type 1/2 collagen are deposited in papillary layer then reticular layer; T cells accumulate and secrete cytokines and increase collagen
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Albinism
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tyrosinase positive or negative; if positive it is present but cannot enter melanocytes; if negative the enzyme is not made; tyrosinase is enzyme in the melanosome membrane; pathway messed up could be tryrosine to DOPA or DOPA to melanin
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Malignant Melanoma Radial Growth Phase
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most reapidly increasing cancer; leading death due to skin cancer; initial phase of invasion; melatocytes proliferate, laterally in epidermis, dermoepidermal junction, in papillary dermis; no metastatic, no tumor cell nodules
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Psoriasis
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inflamed, edematous lesions with silvery white scale; reach apical layers in short time; most common is plaque psoriasis; increased T cells and release TNF; eosinophilia levels over 600/ul; parakeratosis (nuclei in stratum corneum); Langerhans activity; 3-5 days for cell from basal to corneum
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Griscelli Syndrome
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partial albinism of hair and skin; mutation in myosin 5a gene, a molecular motor protein binding to actin and the melanosome when released from microtubule in melanocyte dendritic; binding transfers melanosome to melanocyte dendrite cm; (or a Rab27a or Melanophilin disorder)
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Acne Vulgaris, obstructive type
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plugging of hair follicle by keratin debris; androgen receptors on sebaceous gland stimulate division/matruation of cells and production of sebum; sebum to skin is blocked and follicle distend (comedo), neutrophils; Propionibacterium acnes
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Leprosy
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Langerhans cells use CD1a and langerin to trigger cellular immune response to Mycobacterium leprae (cause of leprosy/Hansen disease); neurologic disease affecting extremities; Schwann cells are target; paralysis/loss of sensation in affected areas; loss of fingers and does
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