Nutrition and sickle cell disease.

The role of protein and calorie deficiency in sickle cell disease remains poorly defined. While such features as growth retardation, impaired immune function, and delayed menarche do suggest a relationship between sickle cell disease and undernutrition, measurement of more direct nutritional parameters in these patients have yielded mixed results. Anthropometric measurements such as skinfold thickness are subnormal in many but not all reports. Serum protein levels are normal, but low values for serum lipids have been reported. Finally, one small study shows an improvement in both growth parameters and clinical course following caloric supplementation. A variety of micronutrient deficiencies have been suggested in sickle cell disease. Numerous case reports describing an exacerbation of the chronic anemia that was reversed by folic acid therapy led to routine folate supplementation. More recent studies have shown, however, that clinically significant folic acid deficiency occurs only in a small minority of sickle cell patients. Clearly, more work is necessary to define the cost/benefit ratio of routine folic acid supplementation. Pharmacological amounts of vitamin B6 and certain of its derivatives possess in vitro antisickling activities. Nevertheless, a small clinical trial failed to demonstrate any consistent hematologic effects of B6 supplementation. Several reports indicate that vitamin E levels are low in sickle erythrocytes. Since these abnormal red cells both generate excessive oxidation products and are more sensitive to oxidant stress, and because oxidants appear to play a role in ISC formation, vitamin E deficiency could well be linked to ISC formation and hemolysis. Small clinical trials, however, have again failed to produce a clear hematological response in sickle cell anemia. The role of zinc in sickle cell disease has received considerable attention. Though studies are generally small, most do support a relationship between sickle cell disease and zinc deficiency. Etiologic associations between zinc deficiency and such complications of sickle cell disease as poor ulcer healing, growth retardation, delays in sexual development, immune deficiencies, and high ISC counts have all been suggested. Most of these studies need further corroboration. Iron deficiency is now known to be a relatively common occurrence in sickle cell anemia, especially in children and pregnant women. The theoretical benefits of concomitant iron deficiency and sickle cell anemia remain to be proven in a controlled clinical trial.(ABSTRACT TRUNCATED AT 400 WORDS)

Parenteral nutrition at home for 5 years via arteriovenous fistulae. Supplemental intravenous feedings for a patient with severe short bowel syndrome.

On March 26, 1970, a 33-year-old male suffered intestinal infarction which required total enterectomy and duodeno-transverse colostomy. Nutrition was maintained in the hospital by daily parenteral feeding for 2 months postoperatively, after which parenteral feedings were decreased and stopped for long periods. Various oral dietary regimens failed to provide adequate nutrition, and the patient lost 40 kg and became severely malnourished during the next 13 months. In June 1971, supplemental home parenteral nutrition (PN) via an arteriovenous fistula was instituted on a 3 or 4 nights per week basis. The patient's weight and strength increased markedly after institution of the home supplemental PN program. The first fistula became occluded after 9.5 months of home PN use and subsequent successive fistulae have remained patent for 31.3, 8.8, and 5.5 months of use. The patient prepares his own PN fluids at home, using a commercial device for filling plastic intravenous fluid bags. Although several different types of fluid have been used, the current mixture of 25% glucose and 2.75% amino acids with added vitamins, potassium, calcium, magnesium, and insulin plus simultaneously administered lipid emulsion has proven most effective. Only when the patient's low fat, low oxalate diet is supplemented with this parenteral mixture 4 nights each week is he in positive nitrogen, phosphorus, and magnesium balance. However, his negative calcium balance is only partially corrected. There has been no sepsis, embolism, or fistula infection during 5 years of home PN.