Reye’s Syndrome

Agatha M. Thrash, M.D.
Preventive Medicine

Epidemiologic and experimental data support the hypothesis that Reye’s syndrome is a disease of modern civilization caused by multiple rather than a single factor, and not occurring earlier than about 1950. It was reported for the first time in medical literature about 1962.

The diagnosis of Reye’s disease depends on a history of a mild infection, usually a sore throat, runny nose, or other evidence of upper respiratory tract infection, becoming critical in hours, and suddenly ending in death. Other outstanding features are disturbances in consciousness, fever, convulsions, vomiting (unremitting), disturbed respirations, altered muscle tone, and altered reflexes. When any child recovering from a mild viral infection begins to vomit and have a change in mental state, Reye’s syndrome should be suspected. Laboratory tests are helpful to establish the diagnosis, as there are marked elevations in the liver enzymes, SGPT, and SGOT, as well as elevated blood ammonia levels, abnormal activity, and low blood sugar.

Reye’s syndrome is a rare disorder occurring at different rates in different races and ages. The range of ages is from a few months to 14 years, the majority of a11 cases occurring at or just before puberty. Apparently Reye’s syndrome is rare in children under one year of age. Only 8% of children of all ages reported to the Center for Disease Control are black. While white children overall are more susceptible to Reye’s syndrome, black infants are more likely than white infants to get the syndrome in the first few months of life. Among black children under six months who do get the disease, boys are more likely than girls to be affected. White male patients predominate from six months of age on. The mortality rate is high, especially in the older age groups. The death rate remains at about 60%. Those who survive, particularly those under one year old, may be left with nerve damage. The severity of the complications is greater usually in the younger aged children.

The cause of Reye’s syndrome is unknown. Environmental, infectious, and genetic agents are all implicated. It has accompanied a large number of viral illnesses including varicella, retrovirus influenza, parainfluenza, adenovirus, Coxsackie, Epstein-Barr, ECHO, herpes zoster, herpes simplex, poliomyelitis, rubella, rubeola, and vaccinia (1).

It is possible that some feature of the patient’s lifestyle or environment may predispose to a greater likelihood to get a viral infection. Six of fifty-eight cases in one study were associated with chickenpox. Perhaps the most common recurring feature of the environment of persons who develop Reye’s syndrome is that they have had influenza or have had an exposure to it, including immunization with influenza virus. Genetic factors may play some part in the development of Reye’s syndrome, but just that part has yet to be determined.

Since upper and middle class children are involved most often, it would seem profitable to investigate whether the disease is doctor-produced (iatrogenic) disease. Antivomiting drugs as well as flu shots and other immunizations have been considered possible causes. In an epidemic affecting 22 children in Montreal, five had received vaccines within three weeks prior to their hospitalization (2). Polio vaccine virus has also been found in spinal fluid in one victim of Reye’s syndrome (3). The Center for Disease Control found in one series of cases reported to them that fifteen of two hundred and sixty-nine children with Reye’s syndrome had been innoculated with live virus vaccines within 30 days before the onset of the illness. It is suggested that live virus vaccines may in some cases serve as cofactors in the case of Reye’s syndrome.

Of 16 Cases, 81% gave a history of viral infection or contact with a person with a viral infection. Most of the sixteen had taken aspirin or other medications, and seven children had a history of excessive aspirin intake. The Center for Disease Control issued a statement by Dr. Eugene Hurwitz that “an analysis of three studies strongly suggests an association between aspirin and Reye’s syndrome.” Scientists speculate that in susceptible children the drug blocks critical activity of cells, resulting in the release of poisonous chemicals that damage the brain and liver. A list of possible neurotoxins which could cause Reye’s syndrome include ammonia, free fatty acids (especially octeneric and propionic acids), several amino acids, lithocholic acid, and aflatoxin, the toxin from Aspergillus flavus. Pesticides or other toxins may be a part of the cause of this disease.

An attempt has been made to show that there is improper amino acid metabolism, particularly with changes leading to ammonia accumulation. Impaired ability to form urea, an end product of protein metabolism, with subsequent ammonia retention may be a large part of the problem. Low blood sugar is a result of altered carbohydrate metabolism.

At autopsy there have been found marked fatty acid changes in the liver, the brain is swollen, and the kidneys show fatty degeneration in the tiny tubules that make up the kidney. It may be that alterations in the liver come first and the changes in the brain and kidneys come second. A toxin might affect the cells in such a way that the blood becomes imbalanced through the liver dysfunction, causing the changes in the brain and kidneys.

Treatment has been mainly supportive. The best nursing care possible must be provided. Peritoneal dialysis has been tried as have exchange transfusions. The early hopes of success with these measures seem to be waning. Evaluation of results from them indicate they were not helpful. In the treatment of Reye’s disease intensive nursing care is proving to be the most helpful (4). Treatments similar to the kidney packs for polio should be tried, consisting of a large hot pack to the back from the base of the skull to the end of the spine for thirty minutes while three minute hot packs over the lower chest and abdomen are given with alternating one minute cold packs. This thirty minute treatment may be given as often as every three hours. It should be accompanied by a hot foot bath at 110 degrees, kept hot during the thirty minutes, and a cold compress to the forehead and face, kept cold during the treatment. A protein-free, high carbohydrate diet should be used if the patient can eat (probably only fruits and juices).

In some children in whom neurological difficulties developed, they seemed to improve neurologically with the passage of time (5).

1. Menkes, John H. Textbook of Child Neurology, 2nd Edition, Philadelphia: Lea and Febiger, 1980, page 354-355.
2. Pediatrics, February, 1977.
3. Archives of Neurology, April, 1974.
4. Pediatrics 60(5):708-714, November, 1977.
5. Journal of Pediatrics 95 (5, Part 1): 706-711, November, 1979.

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