Multiple cerebral aneurysms in patients with sickle cell disease.

Patients with sickle cell disease appear to have a high incidence of cerebral aneurysm. These aneurysms are not associated with the usual risk factors of hypertension, renal disease, or connective tissue disease. We present two recent cases of patients with sickle cell disease and multiple cerebral aneurysms, review the literature on the association between sickle cell disease and cerebral aneurysms, and discuss a mechanism by which sickle cell disease may lead to the formation of cerebral aneurysms.

The sickle cell trait in relation to the training and assignment of duties in the armed forces: IV. Considerations and recommendations.

The main purpose of the military services is the defense of the country and, at time of war, the maintenance of an aggressive, efficient, and effective offense. The prevention of sickness and disability and the preservation of the health and safety of all troops at all times is necessary, not only for the sake of individuals as individuals, but also for the sake of the successful completion of military missions in the best possible manner with minimal chance of failure. Recruits should be selected, trained, and assigned to duties for which they are best qualified and excluded from special operations that, for them, are peculiarly hazardous. Military services cannot afford to take preventable risks. Major consideration should be given to the maximum good of the greatest number of citizens. Policies should be based on scientific facts. Based on facts that are now known, the author believes that individuals with the sickle cell trait (Hb AS) are at greater risk than those without the trait when engaged in military activities that regularly expose them to hypoxic environments or that require maximum stamina and endurance at the time of exhaustive muscular activities.

The sickle cell trait in relation to the training and assignment of duties in the armed forces: III. Hyposthenuria, hematuria, sudden death, rhabdomyolysis, and acute tubular necrosis.

Although the sickle cell trait (SCT) is usually a benign and innocuous carrier state or condition rather than a disease, those with the trait are capable of developing any and all types of vascular occlusive lesions that have been observed in patients with sickle cell anemia. Obstructive vascular lesions in individuals with SCT occur infrequently, but when they do occur they are disabling and may be life-threatening. Disabilities attributed to in vivo sickling have the potential of seriously impeding the success of military missions. When selecting recruits to be trained and assigned to special operations, consideration should be given to hyposthenuria, the possibility of hematuria and to exercise-induced syndromes. Exertion to the point of exhaustion in previously healthy individuals with SCT may cause sudden death, rhabdomyolysis, and acute tubular necrosis. In vivo sickling of erythrocytes is a superimposed and late contributory and complicating factor of exertional syndromes.

The sickle cell trait in relation to the training and assignment of duties in the armed forces: II. Aseptic splenic necrosis.

Well-documented information gleaned from the world's literature reveals that in vivo sickling of erythrocytes and vascular occlusive lesions involving the spleen have occurred in individuals with the sickle cell trait (SCT) while flying in unpressurized airplanes or when exposed to hypoxic environments in mountains at intermediate altitudes. The clinical and anatomical manifestations of splenic infarcts are described. Individuals without the trait do not develop splenic infarcts during or following exposure to ambient hypoxia.

The sickle cell trait in relation to the training and assignment of duties in the Armed Forces: I. Policies, observations, and studies.

Opinions differ widely concerning the risks assumed by individuals with sickle cell trait (SCT) while engaged in military activities that involve exposure to hypoxic environments and other stress situations. Some claim that there is no scientific evidence of greater risk to those with SCT than to those without it, and that any and all restrictions are unfounded and unjust. Others believe that the success of military missions with the least possible risk to the health and safety of individuals are major concerns and that restrictions on the selection, training, and assignment to duties are necessary. Clinical observations indicate that in vivo sickling of erythrocytes in individuals with the SCT may occur in association with cardiac, pulmonary, and other primary diseases. Past scientific investigations have been inadequate and the conclusions drawn have been conflicting. Future policies will be based on information from new investigations combined with facts revealed in the literature and from retrospective studies of the experiences of those with and without the SCT while on duty in the Armed Forces.

Sickle cell centers of tomorrow. Part I: Regional information centers.

Regional sickle cell organizations are needed to assemble, catalogue, and distribute information, to maintain teaching programs, to serve as referral agencies, and to aid individuals with sickle cell hemoglobinopathies in obtaining maximal benefit from existing community services. The governing body should be representative of the entire area. More than half of the voting members should be adults with the sickle cell trait or sickle cell disease and/or their relatives. A suggested minimal staff could consist of a secretary-librarian and a director. The usefulness of the "Center" to patients, especially at the time of their recurrent painful episodes, could be greatly extended by the maintenance of a 24-hour telephone answering service. Funding of the "Regional Sickle Cell Information Center" by the United Way is recommended.

Radiographic changes of the skull in sickle cell anemia.

Skull radiographs of 194 patients from 4 months to 55 years old with sickle cell anemia revealed porous decreased bone density in 25%, widening of diploë associated with a relative decrease in the width of the outer table in 22%, and vertical "hair-on-end" striations in 5%. The youngest patient with vertical striations was 5 years old and the oldest was 39. Serial examinations in 60 patients revealed no decrease of the skull width nor disappearance of the striations with age.

Primary laboratory medicine.

A list of laboratory tests which should be available in a primary care facility is presented. The primary physician should be able to perform or obtain reports of blood smears, urinalysis, hematocrit, white blood counts, sedimentation and prothrombin information during the patient's visit. In general, those tests that are not practical to perform include those that are seldom used, that require extended time for performance, that are complicated and require special technical skills and that demand expensive and elaborate equipment and unstable expensive reagents. Equipment recommended for a primary laboratory is also listed. Tests to be run on each individual patient should be selected carefully because of the cost factor as there are limited financial resources for health care delivery.

Counseling the patient with sickle cell disease about reproduction: pregnancy outcome does not justify the maternal risk.

PIP: A retrospective study of 35 HbSS and 15 HbSC pregnant patients was undertaken to illustrate the hazards to both mother and child of gestations associated with these hemoglobinopathies. It was found that these patients experienced a 10% to 14% maternal mortality rate and a 51% to 55% perinatal mortality rate. The fact that the mother, due to her illness, cannot care for the child, coupled with the poor medical health that the offspring themselves have if they too inherit the homozygous gene, provides additional support to the concept that some form of relief should be offered these women. Based on the figures of this study and those of others, aggressive genetic counseling to the women is advocated including liberal surgical sterilization and primary abortion in certain cases.^ieng