3.20 NIELL Iron Overload Flashcards

1
Q

Why is Fe overload bad

A

-Generated Hydroxyl radicals

–lipid peroxidation, fibrosis, carcinogenesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Alcohol and Iron

A

-30% alcoholics have Fe overload

–Possible folate def increases iron absorption

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Thalassemia and IRON!!!!

A

-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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Hereditary Hemachromatosis

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Hemachromatosis Clinical Symptoms

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Hemachromatosis Lab Findings

A

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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Hemachromatosis Treatment

A
  • 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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Results of Treatment and what will not improve??

A
  • General Health improves
  • Hyperpigmentation lessens
  • Arthalgias may disappear
  • DIABETES will NOT improve
  • Stop cirrohosis of liver
  • If treated before cirrhosis, hepatoma will not develop
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Screen for Hemachromatosis

A
  • 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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Folate Deficiencies

A
  • Body stores 10-12mg (small) so def can develop rapidly/3-4 months
  • Absorption is duodenum and jejunum
  • Main foods: liver, greens and yeast
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Candidates for Folate Supplement

A

People who are/have:

  • Pregnant/lactating
  • Increased RBC turnover (hemolytic diseases)
  • Exfoliative dermatitis
  • Drugs: methotrexate
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Impaired absorption of Folic Acid

A

Tropical Sprue

Regional Enteritis

Resection of small intestines

Seen most in: elderly, poor and alcoholics

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Treatment of Folate Deficiency

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Clinical Features of Megablastic Anemias

A
  • 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)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Lab Values of Megablastic Anemias

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

BOOK EXPLANATION OF LAB VALUES

A
17
Q

Absorption of B12

A
18
Q

B12 Nutritional Aspects

A

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

Absorption site: Ileum

Sources:Animal products, Milk, Eggs and Cheese

19
Q

B12 Def Gastric Disorders

A

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
Q

Intestinal Disorders and other causes of B12 deficincy

A

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
Q

Main differentiation between B12 and Folate Deficiencies?

A

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
Q

PERNICIOUS ANEMIA

A
  • 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
Q

OTHER AUTOIMMUNE DISEASES ASSOCIATED WITH PA

A
  • Auto thyroid disorder
  • Type 1 diabetes
  • Hypoparathyroidism
  • Addison Disease
  • Ulcerative colitis

Vitiligo

-Aquired agammaglobulinemia

24
Q

Schilling Test

A

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