3.20 NIELL Iron Overload Flashcards Preview

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Flashcards in 3.20 NIELL Iron Overload Deck (24):
1

Why is Fe overload bad

-Generated Hydroxyl radicals

--lipid peroxidation, fibrosis, carcinogenesis

2

Alcohol and Iron

-30% alcoholics have Fe overload

--Possible folate def increases iron absorption

Clinical: skin pig, hypogonadism, glucose intolerance, Most Iron in kupffer cells vs hepatocytes

3

Thalassemia and IRON!!!!

-Pts receiving transfusion recieve 200-300ml/kg/year

Clinical problems:

-1st decade with HEPATOMEGALLY (enlarged liver)

-Lack of sexual development (OH NO!!!)

-2nd decade: Cardiomegally (bad) Treatment: Dextroferramine: Fe chelator

4

Hereditary Hemachromatosis

Mutation of HFE gene (Auto Recessive w/ reduced penetrance)

-Result: low hepcidin regardless of iron concentration and iron accumulation develops over years b/c absorption from GI tract even with high iron conctration

5

Hemachromatosis Clinical Symptoms

-Liver Function abnormalities                     -Arhtralgias

-Weakness and lethargy                            -Impotence in men

-Skin hyperpigmentation                           -Electrocardiographic changes

-Diabetes mellitus                                      -Hepatomas (malignant tumor of liver) increase 20 fold

-Present 30-50 yrs old

6

Hemachromatosis Lab Findings

Normal: WBC, Hematocrit and Platelets

Abnormal:

-Serum Iron .180microg/dl

-Iron binding capacity: normal to low (transferrin is being used up)

-Saturation.62% -Serum Ferritin . 800microg/liter

-Liver biopsy: increased iron

7

Hemachromatosis Treatment

-Phlebotomy: bleed the bastards

-Aim to keep ferritin <50ng/ml

Diet Restrictions:

-Raw shell fish

-less fat and animal protein

-avoid vit c (increase absorption)

-avoid alcohol (never)

8

Results of Treatment and what will not improve??

-General Health improves

-Hyperpigmentation lessens

-Arthalgias may disappear

-DIABETES will NOT improve

-Stop cirrohosis of liver

-If treated before cirrhosis, hepatoma will not develop

9

Screen for Hemachromatosis

-Genetic testing of family to see if they have mutation

-Having mutation does mean pt will develop disease

-Insurance companies do not want to pay for and not much evidence supports this

10

Folate Deficiencies

-Body stores 10-12mg (small) so def can develop rapidly/3-4 months

-Absorption is duodenum and jejunum

-Main foods: liver, greens and yeast

11

Candidates for Folate Supplement

People who are/have:

-Pregnant/lactating

-Increased RBC turnover (hemolytic diseases)

-Exfoliative dermatitis

-Drugs: methotrexate

12

Impaired absorption of Folic Acid

Tropical Sprue

Regional Enteritis

Resection of small intestines

Seen most in: elderly, poor and alcoholics

13


Treatment of Folate Deficiency


Give 1mg/day of folic acid orally

-Need to be sure it is not B12 def (give B12 and folate together if unsure) b/c of neuropathy

 

14

Clinical Features of Megablastic Anemias

-Insidious onset: gradually progressive symptoms

-Mild Jaundice: excess breakdown of hemoglobin resulting from increased ineffective erythropoiesis in the BM

-Glossitis: Beefy red tongue

-Angular stomatitis: corners of mouth

-Weight loss (symptoms of malabsorption)

-Purpura: from thrombocytopenia (less common)

15

Lab Values of Megablastic Anemias

PANCYTOPENIA

-WBC: low             -MCH ([Hgb] per RBC): high, cytoplasm keeps dev but cells are not dividing

-Hgb: low               -RDW: high, variation in shape and size

-Hct: low                -Retic Count: low, new RBC are not being produced

-MCV**>98fL (macrocytic)    -LDH and Bilirubin: High, Marrow cell breakdown

16

BOOK EXPLANATION OF LAB VALUES

A image thumb
17

Absorption of B12

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18


B12 Nutritional Aspects


Body Stores 2-3mg, def takes 2-4 yrs to develop

Absorption site: Ileum

Sources:Animal products, Milk, Eggs and Cheese

19

B12 Def Gastric Disorders


Total Gastrectomy

Blind Loop Syndrome

Zollinger Ellison Syndrome: Gastric producing tumor in pancreas secretes HCL prevents transfer of Cbl from R binders to IF

20

Intestinal Disorders and other causes of B12 deficincy


Intestinal:

-Ileal Resection

-Regional enteritis

-Cbl malabsorption from hypothyroidism or drugs

-Tropical Sprue

Other:

-pancreatic insufficiency: lack of transfer to IF

-Dietary: fucking vegans (rare)

21

Main differentiation between B12 and Folate Deficiencies?

 


B12 have Neurological disorders

EARLY:                                                                             

-Parathesias of hands and feet            

-Somulence and dementia                                                 

-Descreased vibritory and proprioception

-Loss of position sense (in dark)

Late:

 -Spastic paralysis from demyelination of dorsal and lateral columns - not reversible

-"Combined system disease"

 

22


PERNICIOUS ANEMIA

-Cause by auto immunoe attack on the gastric mucosa leading to atrophy of the stomach

-Wall of stomach thins, with plasma cell and lymphoid (CD4)  infiltrate of the lamina propria

-Intestinal metaplasia may occur

-SECRETION OF IF IS ABSENT (B12 DEF)

-Antiparietal cell abs in 90% of PA and 60% of atrophic gastritis

-Intrinsic factor abs found in 50-70% of PA

 

23

OTHER AUTOIMMUNE DISEASES ASSOCIATED WITH PA


-Auto thyroid disorder

-Type 1 diabetes

-Hypoparathyroidism

-Addison Disease

-Ulcerative colitis

Vitiligo

-Aquired agammaglobulinemia

24


Schilling Test


PART I:

-Drink cyanocobalamin, 24hr urine collection, at 2 hrs give 1000microg of IM b12, measure in urine

PART II:

-Give 60 mg of hog intrinsic factor with radioactive B12

PART III:

Repeat after 14 days of antibiotics