27 May 2009

Acute Coronary Syndrome



Acute Coronary Syndrome
By:Rich Derby, Lt Col, USAF
MGMC Family Practice Program

Objectives

* Define & delineate acute coronary syndrome
* Review Management Guidelines
* Review secondary prevention initiatives

Scope of Problem
Expanding Risk Factors
* Smoking
* Hypertension
* Diabetes Mellitus
* Dyslipidemia
* Family History—event in first degree relative >55 male/65 female
* Age-- > 45 for male/55 for female
* Chronic Kidney Disease
* Lack of regular physical activity
* Obesity
* Lack of Etoh intake
* Lack of diet rich in fruit, veggies, fiber

Acute Coronary Syndromes
Similar pathophysiology
Similar presentation and early management rules
STEMI requires evaluation for acute reperfusion intervention
* Unstable Angina
* Non-ST-Segment Elevation MI
* ST-Segment Elevation MI

Diagnosis of Acute MI
STEMI / NSTEMI
Diagnosis of Angina
* Typical angina
* Atypical angina
* Noncardiac chest pain

Diagnosis of Unstable Angina
* Patients with typical angina - An episode of angina
* Patients not known to have typical angina
Unstable Angina
Acute Management
Evaluation
Time Sensitive
Chest pain suggestive of ischemia
Immediate assessment within 10 Minutes
Initial labs and tests
Emergent care
History & Physical
Focused History
Targeted Physical
ECG assessment
Normal or non-diagnostic EKG3
Cardiac markers
Prognosis with Troponin
Mortality at 42 Days
Risk Stratification
Cardiac Care Goals
STEMI cardiac care
Fibrinolysis indications
Absolute contraindications for fibrinolysis therapy in patients with acute STEMI
Relative contraindications for fibrinolysis therapy in patients with acute STEMI
STEMI cardiac care
Comparing outcomes
Medical Therapy
Additional medication therapy
STEMI care CCU
Unstable angina/NSTEMI cardiac care
Probable ischemic symptoms
TIMI Risk Score
ACS risk criteria
Moderate to high likelihood of CAD
High Risk ACS
Invasive therapy option UA/NSTEMI
Conservative Therapy for UA/NSTEMI
Secondary prevention behavioral intervention
Secondary prevention cognitive
Medication Checklist after ACS
Summary

Acute Coronary Syndrome.ppt

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25 May 2009

Inborn Errors of Metabolism



Inborn Errors of Metabolism
By:Robert D. Steiner, MD
Associate Professor, Pediatrics and Molecular and Medical Genetics
Head: Division of Metabolism, OHSU

Inborn Errors of Metabolism
* IEM as a group are not rare: occur 1 in 5000 births collectively
* Often treatable if diagnosed
* Most difficult task for clinician is to know when to consider IEM and which tests to order for evaluation
* Don’t be fooled--other diagnoses like sepsis, ICH, pulm. hem. may accompany IEM
* Clues to presence of IEM may often be found in FH

Metabolic Diseases Which Can Present in Crisis
“Stumbling Blocks” in Diagnosing Inborn Errors of Metabolism
* Signs and symptoms are often nonspecific
o Routine childhood illnesses excluded 1st
o Inborn errors considered only secondarily
* Unfamiliarity with biochemical interrelationships/ diagnostic tests
o Inappropriate sample collection
o Inappropriate sample storage
* Every child with unexplained . . .
o Neurological deterioration
o Metabolic acidosis
o Hypoglycemia
o Inappropriate ketosis
o Hypotonia
o Cardiomyopathy
o Hepatocellular dysfunction
o Failure to thrive

. . . should be suspected of having a metabolic disorder

When to suspect an IEM
EFFECT ON OTHER METABOLIC ACTIVITY
e.g., activation, inhibition, competition
Theoretical consequences of an enzyme deficiency.
First Steps in Metabolic Therapy for Inborn Errors of Metabolism
* Reduce precursor substrate load
* Provide caloric support
* Provide fluid support
* Remove metabolites via dialysis
* Divert metabolites
* Supplement with cofactor(s)

Therapeutic Measures for IEM
* D/C oral intake temporarily
* Usually IVF’s with glucose to give 12-15 mg/kg/min glu and at least 60 kcal/kg to prevent catabolism (may worsen PDH)
* Bicarb/citrate Carnitine/glycine
* Na benzoate/arginine/citrulline
* Dialysis--not exchange transfusion
* Vitamins--often given in cocktails after labs drawn before dx is known

Treatment of the Acutely Sick Child
General Therapy
* Maintain vital functions
o Oxygenation
o Hydration
o Acid/Base balance

Specific Therapy
* Treat infection
* High dose I.V. glucose
* Carnitine supplementation

STRIVE TO IDENTIFY PRIMARY METABOLIC DISORDER
TREATMENT OF GENETIC DISEASES
* MODIFY ENVIRONMENT, e.g., diet, drugs
* SURGICAL, correct or repair defect or organ transplantation
* MODIFY OR REPLACE DEFECTIVE GENE PRODUCT, megadose vitamin therapy or enzyme replacement
* REPLACE DEFECTIVE GENE
* CORRECT ALTERED DNA IN DEFECTIVE GENE

Newborn Screening
* PKU - must do on all infants in NICU even if not advanced to full feeds
o Positive--transient HPA, tyr, liver disease, benign HPA, classical PKU
* Galactosemia-
* Hypothyroidism
* Hemoglobinopathies
* Biotinidase def, CAH (21-OH’ase def),
* MSUD

Metabolic Disorders Presenting as Severe Neonatal Disease
What to do for the Dying Infant Suspected of Having an IEM

Inborn Errors of Metabolism.ppt

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Vitamins and Vitamin-Like Substances



Vitamins and Vitamin-Like Substances
By:Eric Niederhoffer & SIU-SOM

* Names and roles - vitamins
* Names and roles - vitamin-like
* Deficiencies and sources -vitamins
* Deficiencies and sources - vitamin-like
* Role in pathways
* Neurotransmitter overview
* Neurotransmitter pathway
* Tetrahydrofolate conversions
* Tetrahydrofolate examples
* B12 pathways

Names and Roles Vitamins
Names and Roles Vitamin-Like Substances
Deficiencies and Sources
Vitamins

A - night blindness
preformed: liver, egg yolk, butter, milk
b-carotene: dark green and yellow veggies
D - ricketts, osteomalacia
milk, fortified food, fish oils, egg yolks, liver
E - neurologic?, hemolytic anemia
veggie oils, nuts
K - bleeding disorders
green leafy veggies, fruits, dairy products, veggie oils, cereals, meats
B1 - beri-beri
seeds, nuts, wheatgerms, legumes, lean meat
B2 - pellagra
meats, nuts, legumes
B3 - pellagra
meats, nuts, legumes
B6 - neurologic disease
yeast, liver, wheatgerm, nuts, beans, bananas
B7 - widespread injury
corn, soy, egg yolk, liver, kidney, tomatoes
B12 - pernicious anemia
liver, kidney, egg, cheese
B9 - anemia
yeast, liver, leafy veggies
C - scurvy
citrus and soft fruits
B5 - none known
yeast, grains, egg yolk, liver


Deficiencies and Sources Vitamins-Like Substances
Choline - rare
whole eggs, liver, beef steak, and soy (lecithin)
Carnitine - unlikely
meat, dairy products, asparagus, wheat grem
Bioflavonoids - none known
fruits, vegetables, tea, coffee, cocoa, wine, beer
Lipoic acid - none known
liver
Coenzyme Q - rare
fruits, vegetables, meats
Inositol - none known
cereal grains
p-Aminobenzoic acid - see B9
liver, rice bran, whole wheat
Glycolysis
TCA
cycle
Glycogenolysis
Role in Pathways
Neurotransmitter Overview

Review Questions

* What are the different names for vitamins A, B1, B2, B3, B5, B6, C, and B12?
* Which pathway depends on vitamin A?
* Which pathways and enzymes depend on vitamin B1, B2, B3, B6 and B12?
* Which pathway and enzyme depends on choline?
* Which pathways and enzymes depend on folic acid?

Vitamins and Vitamin-Like Substances.ppt

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