NUTR1023 Lecture Notes - Lecture 8: Lean Body Mass, Whole Grain, Mixed Nuts

The following CASE REPORT is given:

This 3 1/2-year-old boy was born to nonconsanguineous parents from Chihuahua, Mexico after an uncomplicated pregnancy and delivery. A brother had died at 3 months of age of a "liver problem" after an unexplained coma. The parents and three siblings are well.

At 3 months of age the boy was admitted in coma to a community hospital and suffered a cardiac arrest. Examination revealed hepatomegaly and cardiomegaly and a serum glucose of 15 mg/del (normal, 60-100 mg/dl). The patient eventually recovered and the hepatomegaly resolved. At 6 months of age he developed congestive heart failure after an upper respiratory tract infection and was brought o the medical center of the University of California, Los Angeles. Hepatomegaly and hypotonia were noted on admission. ON day 3 he became lethargic and developed generalized seizure activity and cardiac arrest, but he was successfully resuscitated. Laboratory studies at the time of the arrest revealed a blood glucose level of 15 mg/dl without associated acidosis or ketosis; mild elevation of serum aspartate aminotransferase (SGOT) (337 I.U./liter; normal, 6-36 I.U./liter) and alanine aminotransferase (SGPT) (179 I.U./liter; normal, 10-45 I.U./liter); and hyperammonemia (300 ug/dl; normal <69 ug/dl). Delayed milestones (developmental quotient 66; normal, 100), proximal-muscle weakness, and growth retardation (weight and height below the 3rd percentile) were noted after recovery. Metabolic studies showed normal glucose, galactose, and fructose tolerance, and a normal 10-hour fasting blood glucose, with no increase in lactate, pyruvate, or ketone bodies. The electroencephalogram, brain scan, and chromosomal studies were unremarkable. Also normal were the plasma levels of electrolytes, calcium, phosphorus, magnesium, bilirubin, thyroxine, thyroid-stimulating hormone, and growth hormone. Results of total serum protein determination, serum electrophoresis, cerebrospinal fluid studies, and studies of immune function were also normal. Between acute episodes, serum levels of glucose, ammonia, SGOT, SGPT, and creatine phosphokinase were all normal.

The patient was again admitted with cardiorespiratory arrest after upper respiratory tract infections at the ages of 20, 24, and 33 months. The episodes were associated with liver enlargement, elevations of transaminases to >2000 I.U./liter, and of creatine phosphokinase from 1500 to 33,000 I.U./liter. Maintenance glucose requirements varied from normal (3 mg/kg of body weight per minute) to slightly elevated (5-7 mg/kg/min).

A muscle biopsy specimen contained large amounts of neutral lipids. Liver biopsy also showed severe but nonspecific fatty changes. Abnormal mitochondrial structure, many electron-dense lysosome-like bodies, and dense, laminate, rounded lipofuscin-like particles were evident.

A thirty-two hour fasting study was performed. During the fasting period the patient had no nausea, cramps, or pigmenturia. However, at thirty-two hours he suddenly had a cardiorespiratory arrest characterized by an absence of cardiac electrical activity. He was successfully resuscitated and intravenously glucose was administered.

At the start of the fast his blood glucose level was 92 mg/dl, but by twenty-four hours it had fallen to 66 mg/dl. Plasma triglyceride levels rose from 66 to 126 mg/dl and free fatty acids increased from 0.1 to 2.0 mEq/liter. Serum SGOT rose from 36 to 1450 I.U./liter, with no increase in creatine phosphokinase or aldolase. Ammonia increased from 40 to 134 ug/dl. The most significant finding, however, was the lack of production of measurable ketone bodies.

During the 11th admission, the diagnosis of carnitine deficiency was considered because of fatty changes in the liver and the lack of production of ketone bodies after twenty-four hours of fasting. At that time the patient's height and weight were normal but his developmental quotient was 40, and he had a variety of neuromuscular abnormalities. A computerized axial tomography (CAT) scan of the brain revealed marked enlargement of both lateral ventricles and of the sulci between the cerebral gyri.

QUESTION: For treatment of a systemic carnitine deficiency, the patient was given carnitine supplements and a diet that was high in carbohydrates, low in fat and had 20% of the calories from protein. It was recommended that the fat comes from medium-chain triglycerides. Explain how this diet will help relieve the symptoms of the patient.

Medical History and Physycal E x a m i n a t i o n:
At age 7, Mr. AT suffered a series of seizures, and after a physical e x a m, lab tests, and electroencephalography, he was diagnosed with epilepsy and put on epileptic medication. He has gained 25 lb (11 kg) over the last 2 yr. He complains about excessive postural sway and loss of balance while walking and therefore uses his father’s cane. He has no history of smoking or alcohol consumption, but he has a familial history of diabetes. At a recent physical e x a m his low-density lipoprotein cholesterol (LDL-C) was 178 mg · dl−1, high-density lipoprotein (HDL) was 61 mg · dl−1, fasting glucose was 88 mg · dl−1, and waist circumference was 42 in. (107 cm). He is currently on Carbatrol (carbamazepine) and baclofen.

Diagnosis:
Mr. AT, now 28 yr old, was born prematurely along with his twin sister at 34 wk. His mother experienced a severe infection early in her pregnancy and was hospitalized for several days; upon birth he had an Apgar score of 4. At age 1 he was diagnosed with spastic hemiplegia.

Exercise Test Results:
Because Mr. AT is ambulatory but has balance difficulties, leg cycle ergometry was chosen as the method of aerobic exercise testing. His resting blood pressure and heart rate in a seated position were 134/80 mmHg and 82 beats · min−1, respectively. A small face mask was used to provide an adequate seal. A toe clip on his right side was used to maintain proper foot positioning on the pedals, and an adaptive glove was used to help his right hand grip the handlebar throughout the test. Mr. AT performed a 2 min warm-up without resistance. The leg cycle ergometry test began at 25 W at a cadence of 50 to 60 rpm. The protocol consisted of 2 min stages with an increase of 15 W per stage. The following data were collected during the exercise test.
The exercise test was terminated at 5:15 because of increased hip adductor spasticity and inability to maintain speed of 50 rpm, and by request of the participant because of shortness of breath. No signs of electrocardiogram abnormalities were present during the test. His V.O2 at 5:00 was 16 ml · kg−1 · min−1. The RER was <1.00 and the heart rate and V.O2 failed to reach peak. This was not a valid peak V.O2 test.
Because of the termination of the clinical leg cycle ergometry test, a more functional approach was chosen. The Timed Get Up and Go test was used as a baseline measure. This test used the following protocol.
Test Instructions
1. Starting position: Sit upright against the chair.
2. Stand up from the chair (time start).
3. Move quickly along the line toward the opposite end.
4. Touch the end and pivot back toward the chair.
5. Move quickly all the way back to the chair.
6. Pivot.
7. Sit back down (time end).
The total distance was 10 ft (3.05 m). The test was performed three times. Mr. AT’s scores were 42 s, 38 s, and 40 s. (A score over 30 s suggests a higher risk of falls.) Mr. AT used his assistive device during all testing.

Exercise Prescription:
Using the information presented in the medical history and exercise testing sections and in tables 27.9 through 27.11, develop an exercise prescription that encompasses aerobic, strength, flexibility, and neuromuscular training. Consider the different modes for each of the exercise training domains and the frequency, duration, intensity, and progression of each.

Questions:
1. How would the clinical exercise physiologist best determine the appropriate resistance levels to prescribe for this person?

2. What factors represented in the ICF model should the clinical exercise physiologist take into consideration to increase the adherence level and success of this client?

3. What fall prevention strategies should be incorporated into the exercise prescription for an individual who is at increased risk for falls?

4. Is the assistive device described in this case study appropriate for use by Mr. AT?

5. How should the clinical exercise physiologist incorporate weight loss strategies into the goals for this client?