Biomedical subjects
C S Houston
Publications and source records attributed to C S Houston.
The pressure chamber revisited. A look at the past.
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Operation Everest II.
OEII is an ambitious and complex project which is only possible through the full and enthusiastic support of the U.S. Army Institute of Environmental Medicine. The support staff at the hypobaric chamber will man the chamber controls 24 hours a day throughout the study, and full use of laboratories and equipment has been assured. We will maximize the opportunities for research by involving many other scientists active in altitude research, and with widely differing areas of expertise. Many measurements will be made at extreme altitudes which--at least today--are not possible in the mountain environment. Though some may be possible in a hospital setting, using hypoxic patients, OEII is the only method by which the adjustments to graded induced hypoxia can be studied from their beginning. Thus we will not only shed light on the ability of healthy persons to work on the highest point on earth, but also on the mechanisms which permit survival of many seriously ill patients.
Identical male twins and brother with Cockayne syndrome.
The clinical, radiological, and neuropathological findings in early onset Cockayne syndrome are illustrated in identical twins and their brother. Their appearance of dwarfism with small head and prominent beaked nose strongly resembled that seen in the Seckel syndrome, but unlike patients with that syndrome they had a normal birth weight (for twins), thick cranial vaults, intracranial calcification, and a severe degree of mental retardation. The twins were deaf and blind, with optic atrophy and retinal pigmentation, while their brother had cataracts. Their hands and feet were large in proportion to their small trunk. They had cutaneous sensitivity to any slight exposure to ultraviolet light and severe neurologic problems with incoordination and spasticity. Radiologic findings included microcephaly, a thick cranial vault, a small pelvis, coxa valga, and "ivory epiphyses" in terminal phalanges of hands and feet. Pathologic findings included atrophy of white matter with widespread patchy demyelination, and massive siderocalcific deposits in the brain, particularly in the basal ganglia and cerebellum. While autosomal recessive inheritance is most likely, formally X-linked inheritance cannot be excluded.
The Greig cephalopolysyndactyly syndrome in a Canadian family.
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Radiology of non-immune hydrops fetalis.
A woman presented with polyhydramnios when 33 weeks pregnant, and three weeks later delivered an infant with idiopathic non-immune hydrops fetalis. Ultrasonic scanning before birth showed a severely edematous infant. An abdominal radiograph revealed the classical "Buddha" position of fetal hydrops. The cause of the infant's hypoalbuminemia could not be determined, but the infant responded quickly to intravenous albumin and to thoracentesis for the large pleural effusions. As non-immune hydrops becomes proportionately more common, radiologists should be aware that the diagnosis of "hydrops fetalis" is not synonymous with "erythroblastosis."
High-altitude retinopathy.
Thirty-nine healthy subjects were examined before and after a stay at 5,360 m by ophthalmoscopy and by retinal photography. Twenty of them were also tested for visual acuity, scotomata, and capillary leakage. Vascular engorgement and tortuosity and disc hyperemia were seen in all subjects at altitude and are a "normal" response to hypoxia. Twenty-two (56%) of the subjects had retinal hemorrhages and one showed "cotton-wool spots". These changes are abnormal reactions and are considered high-altitude retinopathy. After maximal exertion on a cycle ergometer, fresh hemorrhages were observed in seven of 34 subjects. The incidence of hemorrhage associated with exercise was significantly greater than predicted. Fluorescein leakage was noted after exercise in eight of 20 persons tested and was associated with exercise-induced hemorrhages. Two persons developed premacular hemorrhages.
The radiation hazard in hospitals--a reappraisal.
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Separating Larsen syndrome from the "arthrogryposis basket".
Many patients with larsen syndrome have been misdiagnosed as having arthrogryposis. Larsen syndrome should be considered in any child with multiple joint dislocations, particularly when the knees are involved. Spinal malalignment of a serious degree was encountered in five of the six patients we report and appears to have been underemphasized previously. Two of the patients had unusual ossicles of uncertain derivation at the elbow. This has been described only five times before.
Separating Pena-Shokeir I syndrome from the "arthrogryposis basket".
The Pena-Shokeir I syndrome is characterized by prenatal onset of growth deficiency, a specific constellation of facial features, multiple ankyloses, camptodactyly, and talipes equinovarus and is almost invariably fatal. A common and perhaps specific radiographic sign is that of subluxations at interphalangeal joints of the fingers, present in four of six patients described here. This autosomal recessive syndrome should be differentiated from arthrogryposis multiplex congenita on the basis of the unusual facial configuration and early lethality, even when interphalangeal subluxations are absent.
Separating monosomy-21 from the "arthrogryposis basket".
An infant with monosomy-21 lived for 20 minutes. The clinical and radiologic findings suggested arthrogryposis, Infants with monosomy-21 have severe intrauterine growth retardation, failure to thrive, arthrogryposis-like findings with restriction of joint mobility, often with joint dislocations, and flexion deformities and malposition of the fingers and toes. Features which help to distinguish it from arthrogryposis include a rather specific craniofacial configuration with a broad base of the nose, an antimongoloid slant of the palpebral fissures, a down-turning mouth shaped like that of a carp, micrognathia, and large low-set ears. The condition is not associated with advanced maternal age. Radiologic findings to date have been quite variable.
High-altitude pulmonary edema in persons without the right pulmonary artery.
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Occasional notes. Varus and valgus--no wonder they are confused.
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Autosomal recessive microcephaly and micromelia in Cree Indians.
In a highly consanguineous, predominantly Cree Indian community in northern Saskatchewan, Canada, 14 similarly malformed babies have been born to eight different mothers since 1953. Six of these infants are reported to assist delineation of the syndrome. The major manifestations of the condition are: Intrauterine growth retardation, perinatal death, marked microcephaly, and severe malformations of the limbs, especially the arms. Elbows are fused, forearms are greatly shortened and usually contain only a single bone, and the hands are very abnormal with only two to four malformed digits. Parental consanguinity, a sex ratio close to one, and a 25% segregation ratio all support autosomal recessive inheritance of this syndrome.
Effects of duration at altitude and acetazolamide on ventilation and oxygenation during sleep.
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Metaphyseal chondrodysplasia, Schmid type. A defect of ultrastructural metabolism: case report.
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Hydrometrocolpos in Kaufman syndrome.
A newborn infant presented with a large abdominal mass, urinary retention and postaxial polyaxial polydactyly. On the basis of intial information from the attending physician, this was at first thought to be a grossly distended bladder, whereas in fact it was a hydrocolpos. The association of hydrometrocolos and postaxial polydactyly is known as Kaufman syndrome.
Effect of acetazolamide on hypoxemia during sleep at high altitude.
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