Dietary management of Type I glycogen storage disease.

The most commonly recognized type of glycogen storage disease (von Gierke's disease) results from deficient glucose-6-phosphatase activity. This enzyme is the last step in the release of free glucose from the liver into the circulation. Thus, the most prominent and life-threatening complication in the illness is severe and often prolonged hypoglycemia, which occurs after the dietary glucose is normally removed from the circulation. With an optimal dietary intake spaced at 2 1/2- to 3 1/2-hour intervals, the blood glucose can be maintained in the normal range during the daytime, but hypoglycemia may occur during overnight fasting. Recent advances in the understanding of the pathophysiology of the illness have led to the use of frequent high-starch feedings during the day and nocturnal intragastric infusions of liquid formulas containing glucose polymers. The liquid formula is infused through either a nasogastric or a gastrostomy tube continuously at night while the patient sleeps. The success of this treatment not only has improved the survival rate but also has corrected the abnormal blood chemistries and generated a more normal rate of growth and development. Because patients with this disease are reaching adulthood in greater numbers, it is necessary for dietitians caring for adults as well as for children to become familiar with the prescribed methods of treatment.

Rapidly declining serum albumin values in newly hospitalized patients: prevalence, severity, and contributory factors.

We had noted that a number of hospitalized patients showed abnormally low serum albumin levels within a few days of admission, although the albumin had been normal at admission. Since this rapid decline in albumin could not be accounted for on the basis of starvation, we hypothesized that the changes were due to the increase in intravascular fluid volume which normally occurs with assumption of the recumbent position. Since albumin is often a nutritional screening parameter in hospitalized patients, it is important to ascertain the incidence of such profound changes in albumin as well as to identify possible causes for this change. A survey of 34 concurrent hospital admissions showed that 28 out of 34 (82%) patients had a decline in serum albumin within 5 days of hospitalization. These 28 patients had a decrease of 0.5 +/- 0.09 g/dl. Twenty-five additional patients were studied in order to elucidate causative factors. Twenty out of the 25 showed a decrease in serum albumin within 5 days of admission (mean decrease 0.5 +/- 0.05 g/dl). Hemoglobins in these patients decreased by a mean of 1.1 +/- 0.34 g/dl (p less than 0.01), but BUN and uric acid levels did not change significantly. The one factor common to all patients with declining albumin values was change in posture. All patients were ambulatory at the time of the initial albumin determination but were on bedrest for at least 6 1/2 hours before the second determination. The findings indicate that most hospitalized patients have significant changes in serum albumin levels which occur with change in posture. If the albumin level is to be used as a nutritional indicator, the patient's position at the time of phlebotomy is essential for accurate interpretation of results.

Nasogastric tube feeding at home: a method for adjunctive nutritional support of malnourished patients.

Many patients with chronic diseases develop malnutrition. Force feeding with either enteral tube of parenteral infusions often succeeds in ameliorating this problem in hospitalized patients. However, after discharge many patients are incapable of sustaining adequate dietary intake. As a consequence, malnutrition may persist or recur. The authors' previous experience using nocturnal enteral tube feedings in patients with glycogen storage disease suggested that malnourished patients also might benefit from enteral tube feedings at home. Fourteen undernourished patients selected for domestic enteral tube feedings clearly demonstrated a tolerance, which included adequate gastric emptying, to the infusions during their hospitalization. They ranged in age from 2 months to 68 yr. Infusion pumps delivered the feedings continuously. At home, 12 patients experienced substantial weight gains. Two maintained their weight while they received intensive chemotherapy for malignancies. Except for the two patients with short bowel syndrome, all patients were weaned successfully to oral feedings after 1 to 3 months. The only apparent complication was possible aspiration pneumonia in a patient with neurological dysfunction. This further experience with domestic enteral tube alimentation indicates that selected patients can be managed effectively, safely and economically with nasogastric nutritional support on an outpatient basis.