Jack B. Shumate

Increased serum creatine kinase after exercise A sex‐linked phenomenon

The effect of 2 hours of exercise on the serum creatine kinase (CK) level was investigated in 11 men and 9 women. The mean increase of CK 24 hours after exercise was significantly greater in men. The relative lack of CK elevation in women may: (1) indicate that female muscle is less susceptible to damage by adverse factors; and (2) explain discrepancies in previous reports.

Carnitine “deficiency” Lack of response to carnitine therapy

A 29-year-old woman had muscle weakness, low concentrations of carnitine in muscle and serum, and abnormally low urinary excretion of carnitine. During a fast, exercise capacity declined, ketone body concentrations rose, metabolic acidosis worsened, and she became hypoglycemic. After treatment with oral carnitine, serum carnitine content returned to normal and blood glucose was maintained during fasting, but ketone body concentrations in blood were even higher. Muscle carnitine content and exercise capacity did not improve. This patient demonstrated features of both systemic and muscle carnitine deficiency, suggesting that the current classification of carnitine-deficiency syndromes is inadequate.

Cyanide insensitive palmitate oxidation in skeletal muscle

Abstract Cyanide insensitive palmitate oxidation is demonstrated in rat gastroc and human biceps using radioisotope labeled palmitate. Similar activity is confirmed in rat liver. The implication for studies of fatty acid oxidation in skeletal muscle as well as for human muscle illnesses is discussed.

Adenylate deaminase deficiency in a hypotonic infant

1. Drummond K: Minimal lesion form of the nephrotic syndrome, in Vaughan V, et al, editors: Nelsons textbook of pediatrics, Philadelphia, 1979, WB Saunders Company, p 145. 2. McLean RH, Forsgren A, and Bjorksten B: Decreased serum Factor B concentration associated with decreased opsonization of Eschericbia coli in the idiopathic nephrotic syndrome, Pediatr Res 11:910, 1977. 3. Anderson DC, York TL, Rose G, and Smith GC: Assessment of serum Factor B, serum opsonins, granulocyte chemotaxis, and infection in nephrotic syndrome of children, J Infect Dis 140:1, 1979. 4. Fikrig SM, Schiffman G, Phillipp JC, and Moel DI: Antibody response to capsular polysaecharide vaccine of Streptococcus pneumoniae in patients with nephrotic syndrome, J Infect Dis 137:818, 1978. 5. AUen RC: Evaluation of serum opsonlc capacity by quantitaring the initial chemiluminescence response from phagocytizing polymorphonuclear leukocytes, Infect Immun 15:828, 1977. 6. Ammann AN, and Pleger RJ: Determination of antibody to pneumococcal polysaccharide with chromium chloridetreated human red blood cells and indirect hemagglutination, Appl Environ Microbiol 24:679, 1972. 7. Giangiacomo J, Cleary TG, Cole BR, Hoffsten P, and Robson AM: Serum immunoglobulins in the nephrotic syndrome. A possible cause of minimal change nephrotic syndorme, N Engl J Med 293:8, 1975. 8. Giebink GS, Schiffman G, Krivit W, and Quie PG: Vaccinetype pneumococcal pneumonia. Occurrence after vaccination in an asplenic patient, JAMA 241:2763, 1979. 9. Ahonkhai VI, Landesman SH, Fikrig SM, Schmalzer EA, Brown AK, Cberubin CE, and Schiffman G: Failure of pneumococcal vaccine in children with sickle-cell disease, N Engl J Med 301:26, 1979. 10, McMillan R, Longmire, R, and Yelenosky, R: The effect of corticosteroids on human lgG synthesis, J Immunol 116:1592, 1976.

Riboflavin-responsive lipid myopathy and carnitine deficiency.

We used riboflavin to treat a patient with lipid myopathy, reduced exercise capacity, intolerance to fasting, and reduced concentrations of carnitine in muscle and serum. Although carnitine concentrations did not change, exercise capacity doubled, and response to fasting improved. Muscle enzyme assay showed that palmityl CoA dehydrogenase activity with and without added flavin adenine dinucleotide (a riboflavin product) was normal. Another riboflavin derivative, electron transfer flavoprotein, could be the site of the defect.

Skeletal Muscle Fatty Acid Oxidation during Early Postnatal Development in the Rat

(1-14C)-palmitate oxidation in rat skeletal muscle homogenates increased only minimally from birth until 15 days of age and then increased more than five times between 15 days and adulthood. In contrast, liver oxidation of palmitate reached adult levels by 4 days of age. Although muscle carnitine concentration, carnitine palmityl transferase activity, and total muscle protein increased during the neonatal period, these changes did not completely parallel the rise in muscle palmitate oxidation. Palmityl-CoA synthetase and palmityl-CoA dehydrogenase activities also did not parallel the rise in overall palmitate oxidation.

Poster Presentations IV Morning Meeting Saturday, April 28, 1979

giography that permits dynamic viewing ofthe vascular flow patterns of AVMs. This technique provided a movielike presentation of the flow data, with an image of the AVM changing from an arterial to a venous phase mass, rather than a static image, as in arteriography. Arteriography visualized the specific arterial and venous channels of the AVM. In contrast, radionuclide angiography offered an overall perspective of the vascular mass, showing major vessels as well as perfusion of the adjacent brain parenchyma. Computerization also permitted multiple flow studies utilizing different projections of the head (e.g., anterior Townes, lateral oblique, vertex, etc.). Dynamicviewing ofthe AVMfrom multiple projections aided in assessing the relative contribution of different feeding vessels which supply the lesion. Radionuclide angiography is an effective, noninvasive diagnostic procedure requiring an intravenous injection of the radiopharmaceutical and approximately 50 seconds for completion of each projection. (8 of 32) with early seimtw and 30 percent (6 of 20) with late seizures had initial EEGs in class IV. All six patients with PLEDs on their initial EEG developed seizures. Only 2.0 percent of the patients (4 of 198) who remained seizure-free following cerebral infarction had EEGs in class IV. Although the majority of the patients who developed postinfarction seizures had EEGs class I, 11, or 111, these data suggest that EEGs should be done on all patients following cerebral infarction and consideration given to anticonvulsants for patients with class IV EEGs.