Continuing lessons from glycogen storage diseases

Article Abstract:

Glycogen storage disorders were among the first inherited metabolic disorders to be identified, since it was possible to microscopically observe the accumulation of glycogen in body tissues obtained at autopsy. The development of more sophisticated chemical analysis, as well as the development of the electron microscope, permitted the more detailed classification of glycogen storage diseases, and now 10 distinct inherited abnormalities of glycogen storage are recognized. In the January 3, 1991 issue of The New England Journal of Medicine, researchers report on the use of liver transplantation in the treatment of Type IV glycogen storage disease. This disorder, also called Andersen's disease and amylopectinosis, results when the deficiency of a particular enzyme changes the molecular structure of glycogen. Rather than a repeatedly branching structure, the glycogen has a more straight structure, which resembles starch, and is far less soluble than glycogen. This insoluble glycogen accumulates not only in the liver, but in muscle and other tissues as well. Death usually results from liver failure early in life. It might be anticipated that liver transplantation would improve survival. However, since the enzyme causing the disorder remains defective in the muscles and the heart, it might also be expected that progression of the glycogen storage disorder would continue in other tissues, and the patient would die of heart failure. It is startling to find that this did not occur in the five long-term survivors of liver transplantation. Not only did the abnormal glycogen storage in muscle not progress, but it actually seemed to abate. In one case, the patient remains alive 73 months after surgery, far longer than he would otherwise be expected to survive. Since all the transplant patients were treated with immunosuppressive drugs, it is not impossible that these drugs exerted a beneficial effect on abnormal glycogen storage in other tissues. However, it is also possible that the normal enzyme being produced in the donated liver in some way favorably affected other organs and tissues as well. Although it is uncertain how this might occur, the observations of these patients may prove to be very important in the development of therapies for patients with many other sorts of enzyme deficiencies. (Consumer Summary produced by Reliance Medical Information, Inc.)

Case studies, Physiological aspects, Surgery, Glycogenosis, Glycogen storage diseases, Metabolism, Inborn errors of, Inborn errors of metabolism, editorial

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Transfer of symptomatic peanut allergy to the recipient of a combined liver-and-kidney transplant

Article Abstract:

The case of a peanut allergy transferred in a liver transplant indicates that the liver has immune cells that can migrate from the liver to other organs. A 22-year-old man with a peanut allergy died of anaphylactic shock after eating a peanut sauce. His liver and one kidney were donated to a man and his other kidney and pancreas were donated to a woman. Three months later, the man developed a rash and difficulty breathing after eating peanuts. Analysis of skin samples revealed cells from the donor as well as his own cells. The woman did not develop a peanut allergy, indicating that the immune cells had come from the donor's liver.

Health aspects, Causes of, Transplantation of organs, tissues, etc., Organ transplantation, Tissue transplantation, Transplantation, Peanuts, Peanut allergy

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