Pathology Final - Kidney

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Pathology Final - Kidney
2011-12-05 18:52:20

Pathology Final
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  1. Adult polycystic kidney disease:
    • Manifests on 30s-40s
    • Autosomal dominant; most common inherited kidney disorder
    • Multiple cysts
    • Often associated with Berry aneurysm of circle of Willis
    • Manifested clinically by hypertension, hematuria, renal mass, progression to renal failure
    • Can be a cause of secondary polycythemia
  2. Renal failure
    • Azotemia (retained nitrogen)
    • Uremia in late stages
    • Acidosis---from accumulation of sulphates, phosphates, and organic acids
    • Hyperkalemia (increased serum sodium)
    • Hypocalcemia
  3. Renal Cell carcinoma
    • Most common renal malignancy
    • Characterized by clear polygonal cells
    • Frequently invades renal veins
    • Triad of flank pain, palpable mass, hematuria
    • Can have secondary polycythemia (due to increased secretion of erythropoietin)
    • Can have ectopic hormone production
  4. Wilms tumor
    • Children age 2-4
    • Large flank mass
  5. Transitional cell carcinoma
    • Most common tumor of the urinary collecting system
    • Hematuria
    • Related to:Cigarette smoking, Aniline dye, Long term cyclophosphamide, Phenactin abuse
  6. Acute post streptococcal nephritis
    • Clinically acute nephritis---fever, nausea, oliguria, hematuria, red cell casts, mild proteinuria (< 1 gram per day), and mild to moderate hypertension, murky brown urine
    • Occurs 1-4 weeks after Group A Beta hemolytic strep infection; more common in children
    • Hypercellularity of glomeruli microscopically; increased acute inflammatory cells, proliferation of mesangial cells
    • 90% of children recover in 2-3 months; 60% of adults recover
  7. Rapidly progressive glomerulonephritis (Crescentic glomerulonephritis)
    • Unknown cause; 25% mortality; 40% proceed to end stage renal disease
    • Crescentic glomerulonephritis----Glomerular basement membrane ruptures allowing entry of inflammatory cells and fibrinogen into the urinary space of Bowman’s capsule--crescents are composed of fibrin, inflammatory cells, and proliferating epithelial cells---ultimately this will scar and hyalinization---these crescents compress glomerular capillaries
    • Several disease processes can cause this form of glomerular disease: Goodpastures---anti-glomerular membrane antibodies, Wegener’s, SLE, IgA nephropathy
  8. Membraneous nephropathy
    • Etiology unknown in most cases; nephrotic syndrome
    • Most common cause of primary nephrotic syndrome in adults
    • Immune complex deposition on the subepithelial side of the GBM--"lumpy-bumpy"
    • Thickened GBM, but more permeable
    • Non-responsive to steroid therapy--progresses to end stage renal disease in 10-15 years
  9. Lipoid nephrosis
    • (minimal change disease, nil disease)
    • Most common cause of nephrotic syndrome in children
    • Unknown etiology
    • On electron microscopy---foot processes fused
    • Responds to steroids--occasionally recurs
  10. IgA nephropathy (Berger’s)
    • Most common clinically recognized glomerular disease in world
    • Can present as nephritic, nephritic or both (mixed)
    • Elevated IgA in blood ; Upper resp infection, Gastrointestinal infection or urinary tract infection in 55%
    • More favorable prognosis in young patients--worse in older adults
  11. Diabetic nephropathy
    • Basement membrane thickening
    • Hyaline arteriolosclerosis causes glomerular ischemia
    • Proteinuria
    • Increased risk of pyelonephritis (infections)
    • Increased risk of papillary necrosis
    • Atheroscleorotic plaques of renal artery and aorta--vascular under-perfusion
    • Kimmelsteil-Wilson nodules (rounded hyalinized areas in glomeruli)
  12. Nephrotic syndrome
    • (massive protein spillage into urine)
    • Lipoid nephrosis, Focal segmental, glomerulosclerosis, Membranous glomerulosclerosis, Diabetic nephropathy, Amyloidosis
    • Features: Massive proteinuria >3.5 g/day, Severe edema, Excessive glomerular permeability, Hypoalbuminemia, Lipiduria, Hyperlipidemia
  13. Mixed nephritic-nephrotic syndrome
    • IgA nephropathy
    • Lupus nephritis
    • Membranoproliferative glomerulonephritis
  14. Nephritic syndrome
    • (bleeding in glomeruli; hypertension)
    • Acute post-streptococcal glomerulonephritis
    • Rapidly progressive glomerulonephritis
    • Goodpasture’s syndrome
    • Features of Nephritic syndrome: Gross hematuria with red cell casts, Oliguria, Mild proteinuria, Uremia, Varying degrees of hypertension
  15. Amyloidosis
    • Deposition of abnormal extracellular fibrillar material in the form of Beta-pleated sheets (large molecule incapable of being digested by the body’s enzymes.
    • Appears like an amorphous bright pink material on H&E stains; apple green birefringence with Congo red stain.
    • May be localized to one organ or tissue; may be systemic—usually fatal—amyloid deposited in blood vessel walls.

    • Examples:
    • Reactive systemic amyloidosis (AA type): Usually caused by chronic inflammatory disorder such as tuberculosis, Hodgkin’s disease, rheumatoid arthritis, Bronchiectasis, or chronic osteomyelitis
    • Myeloma associated amyloidosis (AL type)
    • Hemodialysis associated amyloidosis (AH type)
    • Localized amyloidosis: Alzheimer’s disease (AB type)
  16. Leukocytosis
    • Increase in numbers of circulating WBCs
    • Types: Reactive (secondary) response of bone marrow to infection, Primary—caused by bone marrow disease such as leukemia orlymphoma
  17. Anisocytosis
    Abnormalities in red blood cell size
  18. Poikilocytosis
    Abnormalities in RBC shape
  19. What two things may cause anemia?
    • Increased red blood cell loss: External blood loss, Red blood cell destruction-Hemolytic anemia
    • Decreased red blood cell production: Hematopoetic cell damage from infection, drugs, radiation, or other similar agents, Deficiency of factors necessary for heme or DNA synthesis: Iron, vitamin B12 or folate
  20. What are some common symptoms of hemolytic anemia?
    • abnormal paleness or lack of color of the skin
    • jaundice, or yellowing of the skin, eyes, and mouth
    • dark color to urine
    • fever
    • weakness
    • dizziness
    • confusion
    • intolerance to physical activity
    • enlargement of the spleen and liver
    • increased heart rate (tachycardia)
    • heart murmur
  21. Hereditary spherocytosis
    • Most common cause of hemolytic anemia in US
    • Autosomal dominant; abnormality of spectrin
    • Cells are spherical; reduced deformability; abnormally fragile cells
  22. Sickle cell anemia
    Mutation in Beta-globin coding sequence causing an abnormal form of hemoglobin. Heterozygous patients have the sickle cell trait which has no significant clinical abnormalities.
  23. Sickle cell anemia (homozygous individuals)
    • More than 80% of hemoglobin is abnormal (HbS) causing serious clinical complications: anemia, sickle shaped erythrocytes; recurrent and painful crises; non-healing leg ulcers. Splenic infarcts.
    • Thalassemia---one or more of the genes responsible for the synthesis of Alpha or beta globin chains is abnormal.

    Alpha-thalassemia –deletion of one or more alpha globin genes; range from no clinical abnormalities to fetal death (4 deletions); less severe than Beta thalassemia

    Beta-thalassemia---mutation in Beta-globin gene o Thalassemia minor—heterozygous—mild anemia o Thalassemia major (also called Cooley’s anemia, Mediterranean anemia)—homozygous---severe anemia requiring frequent blood transfusions to sustain life.
  24. Glucose 6 phosphate dehydrogenase deficiency
    • Inherited x linked disorder
    • Reduced ability of RBC to protect itself from oxidative injury (drugs, toxins, infections)
  25. Iron deficiency anemia
    • Microcytic hypochromic anemia due to nutiritional deficiency, malabsorption, chronic blood loss, or increased requirements
    • Labs:  Decreased serum iron  Decreased ferritin  Decreased bone marrow iron stores  Increased total iron binding capacity (TIBC)
  26. Macrocytic anemias
    • Folic acid and B12 deficiencies, chronic liver disease, alcoholism
    • B12 deficiency uncommon except in vegans or in patients lacking intrinsic factor
    • Folate deficiency more common and may be related to anticonvulsant or antimetabolite drug therapy
  27. Anaplastic anemia
    • Hypocellular bone marrow and peripheral pancytopenia (anemia, leucopenia, and thrombocytopenia)
    • May be caused by toxic exposure, radiation exposure, chemicals such as benzene, viral infection, and therapeutic drugs such as chloramphenicol, gold salts, NSAIDS.
  28. Myelophthisic anemia
    • Most often due to marrow replacement by a malignant neoplasm
    • Occasionally due to fibrosis of the marrow
  29. Von- Willebrands disease
    • Most common of hereditary coagulation disorders
    • Autosomal dominant
    • Abnormal platelet adhesion associated with low factor VIII activity
    • Prolonged bleeding time and APTT
  30. Hemophila A
    • ( Factor VIII deficiency; classic hemophilia)
    • Characterized by frequent episodes of hemorrhaging into a major joint
    • Common X-linked disorder
    • APTT is prolonged
    • Lab tests: factor VIII assay
  31. Hemophila B
    • ( Factor IX deficiency, Christmas disease)
    • Less common than hemophilia A ( 1/5 that of hemphilia A)
    • Indistinguishable from Classic hemophilia in clinical features and inheritance
    • APTT prolonged
    • Test: Factor IX assay
  32. Fetal alcholol syndrome
    • Associated with excessive alcohol intake during pregnancy
    • Characterized by facial abnormalities, developmental defects such as microcephaly, atrial septal defect, mental and growth retardation, and other abnormalities.
  33. Prion disease
    • Caused by infectious particles devoid of DNA or RNA; resistant to heating
    • Long incubation period ( used to be categorized as a slow-virus disease)
    • Kuru--caused by ingestion of infected human brain by cannibals of New Guinea
    • Creutzfeldt Jacob Disease (subacute spongiform encephalopathy): Potential hazard to workers in contact with brain specimens, Can be transmitted by corneal transplants, Variant form is mad cow disease from contaminated beef products
  34. Creutzfeldt Jacob Disease (subacute spongiform encephalopathy):
    Potential hazard to workers in contact with brain specimens, Can be
    transmitted by corneal transplants, Variant form is mad cow disease from
    contaminated beef products
    • A prion disease
    • (subacute spongiform encephalopathy)
    • Potential hazard to workers in contact with brain specimens, Can be transmitted by corneal transplants,
    • Variant form is mad cow disease from contaminated beef products
  35. Multiple sclerosis
    • Begins usually between ages 20 and 30
    • Multifactorial etiology---environmental and genetic
    • Highest frequency in people of northern European ancestry
    • Incidence increases with distance from the equator
    • Changes confined to CNS with depletion of the myelin producing oligodendrocytes and focal areas of plaues especiallt in the paraventricular areas. Lymphocytes and macrophages infiltrate the plaques
    • Characterized by exacerbations and remissions
    • Early manifestations include visual and sensory disturbances, weakness of the lower extremities, and possible loss of bladder control
  36. Guillian Barre syndrome
    • Demyelinating disease of peripheral nerves
    • Young adults, often proceeded by viral infection, immunization, or allergic reaction
    • Thought to be autoimmune etiology
    • Manifested clinically be ascending weakness and paralysis starting with the lower extremeties
    • Causes albumino-cytologic dissociation of CSF--markedly increased protein concentration with only a modest increase in cell count
  37. Alzheimer disease
    • Slow progressive intellectual deterioration during the course of several years
    • Loss of recent memory early in course
    • Loss of long term memory causing inability to read, speak, or count
    • Motor problems such as contractions or paralysis
    • Sporadic form presents at age 60 or later; familial form can be present as early as age 40
    • Changes: Generalized cerebral atrophy with moderate neuronal loss, Neurofibrillary tangles and plaques, Hirano bodies (eosinophilic inclusions consisting of actin), Amyloid deposits around blood vessels, Widened sulci
    • NOTE: similar changes are observed with aging; Down syndrome patients over 40 often exhibit Alzheimer findings
    • Neurofibrillary tangles can also be seen with Parkinson’s
  38. Huntington disease
    • Autosomal dominant, progressively fatal disease
    • Clinical abnormalities begin between age 30 and 40
    • Increased numbers of tri-nucleotide CAG repeats
    • Manifested by chorea (irregular dancing-like movements), motor deterioration, and wasting
    • Atrophy of brain, loss of neurons, and gliosis of caudate nuclei
  39. Idiopathic Parkinson disease
    • Usually presents after age 50
    • Neuronal depletion; depigmentation of substantia nigra, Lewy bodies
    • Dopamine depletion
    • Pill-rolling tremors; expressionless face; shuffling gait, slowness of movements
    • Other causes of Parkinsonism include: Trauma (boxers), Drugs and toxins, Encephalitis concurrent with the influenza epidemic 1915-1918
  40. Amyotrophic lateral sclerosis (ALS)
    • Degeneration of upper and lower motor neurons
    • Degeneration of lateral corticospinal tracts and anterior motor neurons
    • Characterized by spasticity, hyper-reflexia, atrophy of upper and lower musculature (denervation of muscles)
    • Onset in early middle age; rapid course leading to death from respiratory failure in 1-6 years
    • Most common form of motor neuron disease
  41. What is the 2nd most common form of malignancy in children? (leukemia is number one)
    CNS tumors
  42. Glioblastoma multiforme
    • most common primary intracranial neoplasm
    • Peak incidence in late middle age
    • Highly malignant; rapidly growing
    • Most often in cerebral hemisphere
  43. Meningioma
    • benign slow growing tumor
    • More frequent in women
    • Usually occyurs after age 30
  44. Medulloblastoma
    • Highly malignant tumor of cerebellum in children
    • One of the more common childhood malignancies