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PubMed1985

Reye syndrome.

Reye syndrome has emerged as the quintessential example of an acute metabolic encephalopathy. The clinical presentation is quite stereotyped in most instances permitting rapid, accurate diagnosis and early therapeutic intervention. Intoxications and certain inborn metabolic errors may mimic Reye syndrome. All patients with a recurrent episode should be investigated thoroughly for evidence of a metabolic disorder associated with an enzyme deficiency. Notable in this regard are inborn errors affecting organic acid, ammonia, and carbohydrate metabolism. The mitochondrial disturbance in Reye syndrome is well documented but the pathophysiologic sequence linking the antecedent viral illness to the mitochondrial injury remains obscure. Recent identification of a spontaneous Reye-like illness in mice that is associated with a coronavirus infection may provide an opportunity to investigate this initial phase of the pathophysiologic sequence. The primary cerebral insult presumably derives from insufficient substrate availability and results in massive cytotoxic cerebral edema. Treatment revolves around the continuous infusion of hypertonic glucose and intermittent infusion of hypertonic mannitol. Management is designed to attenuate or avoid the various compounding metabolic insults during this critical period when the child is metabolically crippled. In 1963, the disorder was considered to be rare and almost irreversibly fatal. Today, the disorder is recognized to be more common, and the outcome is very satisfactory in 85 to 90 per cent of the cases. The role of aspirin remains very controversial. A number of studies suggest an association between this potential mitochondrial toxin and Reye syndrome, but a causal relationship has not been established. Until better understood, it seems advisable to avoid use of aspirin in children exhibiting symptoms suggestive of Reye syndrome.

Anesthesia, General↗

PubMed1985

Leprosy in a mangabey monkey--naturally acquired infection.

Naturally acquired leprosy was detected in an otherwise normal "sooty" mangabey monkey (Cercocebus atys). This animal was imported from West Africa in 1975 and developed clinical symptoms of leprosy in 1979. Histopathologic findings were those of subpolar-lepromatous to borderline-lepromatous leprosy in the Ridley-Jopling classification. The disease was progressive, with crippling neuropathic deformities of the hands and feet. The disease regressed under specific therapy. The etiologic agent was identified as Mycobacterium leprae by the following criteria: invasion of nerves of host, staining properties, electron microscopic findings, noncultivable on mycobacteriologic media, DOPA-oxidase positive, lepromin reactivity, infection patterns in mice and armadillos, sensitivity to sulfone, and DNA homology. We believe the animal acquired the disease from a patient with active leprosy. The mangabey monkey offers promise as a primate model for leprosy, and adds a third reported species to animals with naturally acquired leprosy.

Animals↗

PubMed1985

Position of the American Heart Association on research animal use.

Millions of Americans today are healthy, and other millions are alive, because of advances in the prevention and treatment of heart disease. Death rates from the major forms of heart disease have declined steadily since about mid-century, and the decline is continuing. Most recently, between 1972 and 1982, the death toll from cardiovascular disease declined 28 per cent. The decline is largely related to changes in life style and development of methods of treatment, many of which are based upon animal experimentation. These events encourage medical scientists to believe that most heart attacks, strokes and hypertension can ultimately be prevented or their onset can be deferred so that they do not cripple or kill people prematurely.

American Heart Association↗

PubMed1985

A combination pelvic osteotomy for acetabular dysplasia in children.

Several surgical procedures have been devised to increase acetabular coverage of the femoral head in children with dysplasia of the hip. In this report we describe an acetabuloplasty that combines the key aspects of the Pemberton and Salter osteotomies. It has been used at the Los Angeles Unit of the Shriners Hospital for Crippled Children since the late 1960's. To assess the results of this combination procedure, fifty hips in forty-four children were evaluated at an average of six years postoperatively. The average age at operation was 7.3 years, and 62 per cent of the patients had had prior surgery. Clinically, thirty-two hips in which there had been no or slight symptoms preoperatively remained unchanged, twelve that had had preoperative limitations improved, and six showed some deterioration in terms of slight loss of motion, mild pain, and a limp. Roentgenographically, acetabular dysplasia (as measured by the acetabular index and by the center-edge angle of Wiberg) improved in more than 90 per cent of the hips. The roentgenographic results were comparable with those obtained by innominate or pericapsular osteotomy. The combination osteotomy has the advantages of both the Pemberton procedure and the Salter operation and proved to be an excellent surgical procedure for older children whose acetabular development did not progress as well as was expected.

Acetabulum↗

PubMedDate not supplied

Alzheimer's disease: a practical, psychological approach.

Alzheimer's Disease affects approximately two million people. It is a crippling, organic brain disorder that causes loss of recent memory, intellectual deterioration, unpredictable behavioral changes, and personality deterioration. The fourth leading cause of death among the elderly, it also affects younger people. The disease has two victims, the Alzheimer patient and the caregiver. Caregivers often experience shame, embarrassment, denial, frustration, anger, depression, and guilt as they care for an Alzheimer patient. This paper provides information about the disease and it's manifestations, along with practical suggestions to help both the Alzheimer patient and the caregiver.

Adult↗

PubMed1977

Regional services for children and youth with diabetes.

A regional diabetes program for children and youth comprising outreach clinics, frequent routine and emergency telephone contact, and a camping program was developed within the State Children's Medical Services (Crippled Children's) Program. The pediatric nurse specialist in diabetes served as the pivotal professional in the education and supervision of patients. Cost benefit was calculated from estimated hospital days prevented based on average length of admissions at diagnosis (14 days) and hospital days in the year prior to referral of those with established diabetes. During the first year 369 hospital days were prevented, for an estimated savings of $65,000; the second year 547 hospital days were avoided, a value of $96,000. Total annual program costs to the state and private patients (including camp fees) were $36,000. Although hospital costs are an apparent and useful reference to justify funding, the main value of the program must be in the prevention of secondary physical and emotional disability. This requires continuous commitment and availability of persons who know the patient and family, who can interpret information related to metabolic control of diabetes, and who are able to influence the family and community to respond appropriately to the youngster's needs.

Adolescent↗

PubMed1979

Hip disease of Mseleni.

Mseleni in Northern Zululand is a remote geographically isolated improverished district in which about 2,000 persons suffer from a crippling joint disease similar to dysplasia epiphysealis multiplex and spondyloepiphyseal dysplasia. Eleven per cent of the men and 39% of the women in young adult life are seriously disabled and, with concurrent osteoarthritis, 90% of the population are grossly incapacitated in old age. Affected and unaffected control areas have been compared. Analysis of the climate, dietary factors, social taboos and soil and water does not implicate these as causes. Genetic studies, despite considerable field work, sociological surveys and laboratory investigations have not provided the answer. Despite a high incidence in the Mseleni area no disturbance of blood group percentages could be determined. The disease had dissimilarities from Kashin Beck disease in which fusarium fungus allegedly causes small joint polyarthritis. Fusarium sporotrichiella has not been grown in subtropical Zululand and thus could not be a causative agent. Mseleni disease also differs from Handigodu disease in Karnataka India, in which the hips are also destroyed in 80% of cases in the acute form of this disease which is brought on by environmental factors related to subtropical climate, low animal protein intake and intake of a variety of possibly toxic products of vegetable origin. Investigation of stored groundnuts in the affected districts has not revealed a fungus toxin in food which can as yet be implicated. A multifactorial genetic and environmental cause appears probable.

Adolescent↗

PubMed1979

Sensory feedback in a myoelectric upper limb prosthesis: a preliminary report.

Upper limb prostheses are often rejected because they do not provide the sensory feedback available from a normal hand. A system for providing sensory feedback has been developed at the University of New Brunswick for use with the three-state myoelectric controls prepared in the Bioengineering Institute there. Strain gauges mounted on the forefinger of an electric hand provide information which is processed electronically to cause a tingling sensation in the patient's stump, proportional in its intensity to the pinch force in the finger. This system has been used by a patient at the Ontario Crippled Children's Centre in Toronto, since June 1976. She uses it consistently with great satisfaction and enthusiasm. It gives her a sense of competence and confidence she does not have without it.

Arm↗

PubMedDate not supplied

The function of the toes in walking, jogging and running.

The foot has been studied in walking, running and jogging using high speed cinema photography in the laboratory for gait analysis in the Shriner's Hospital for Crippled Children, San Francisco. During running, as well as in walking, the extrinsic toe flexors restrain progression of the tibia. The extrinsic extensors accelerate the tibia over the fixed foot. The intrinsic toe muscles function from mid-stance to lift-off when the subject runs on his toes. In sprinting, the intrinsics are active throughout the weight-bearing phase.

Gait↗

PubMed1979

Two-year review of hand infections at a municipal hospital.

Although an unromantic topic, the management of hand infection can tax the patience and skill of the modern day surgeon. The addition of antibiotics to the surgeon's armamentarium have made the job easier than in the days of Kanavel and Koch but has not altered the basic surgical principles of elevation, immobilization and properly planned drainage. Awareness of hand anatomy in relation to infection and awareness of special categories of problems are necessary in preventing crippling deformities in one of our most precious possessions--the hand.

Animals↗

PubMed1979

Poliomyelitis vaccination benefits--versus risk.

Paralytic poliomyelitis is a constantly rising problem in the developing world. It may take an insiduous endemic "infantile paralysis" behaviour exacting a high toll in the first few years of life, as in Ghana or Burma but on the other hand it may take the form of sudden extensive outbreaks of paralytic disease as in Argentina, Mexico or Malaysia. The developed world has controlled the disease by effectively immunizing a very high proportion of their populations, but those who have not been vaccinated are at risk even in countries with very high coverage, as has been noted in the Netherlands, Sweden, United States of America, etc. There is no reason to have a crippled paralytic child (or adult). Both the live and killed vaccines have been repeatedly shown to be safe and effective. The minute risk incidental to vaccination is more than one hundredfold smaller than the risk from the disease, not only in the developing world but in the developed world as well. Therefore, the question of which vaccine to use is of far less relevance than of how to increase effective coverage with any available vaccine. This does not mean that vaccine control should be relaxed. A health respect should be maintained for the polioviruses used as vaccine sources and great care must be exercised by those undertaking the manufacture or the administration of vaccine.

Child↗

PubMed1979

Recent advances in hemophilia.

Classical sex-linked hemophilia (Hemophilia A) has been described as due to deficiency in the synthesis of Factor VIII procoagulant activity (VIII:C). The availability of immunological techniques provided the means of identifying Factor VIII-Related Antigen(VI-IIR:Ag) detectable by rabbit antibodies to F VIII, which is distinct from VIII:C detected by human anti-F VIII available from multitransfused patients. Hemophilia A is lacking in VIII:C but not VIIIR:Ag. Recently, a third function of the F VIII "complex" was discovered with the help of ristocetin (von Willebrand's Factor, VIIIR: RCo). This activity is reduced in von Willebrand's syndrome. Estimation of the titers of VIII:C and VIIIR:Ag provides a method for more accurate detection of hemophilic carriers. Newly available chromogenic substrates perhaps will give rise to more simplified assays of VIII:C. The development of cryoprecipitates and stable lyophilized concentrates of F VIII has greatly simplified and intensified maintenance therapy, and has opened a new era in treatment. Prophylactic therapy has been shown to be very helpful in certain "high risk" cases. The impact and benefits of home care and self-administration has been tremendous. However, the varying quality of cryoprecipitates and the high cost of more purified concentrates are still stumbling blocks in treatment regimes. Other problems exist. Spontaneous bleeding, especially central nervous system bleeding, account for the majority deaths by haemorrhage. Inhibitor kinetics have been well characterized. It is clear that there exists "low" and "high" responders. For the "high" responders, plasmapheresis, immunosuppressives and the infusion of Factor IX concentrates have been utilized with varying success. The prevention of hemophilic arthropathy and its progression by maintenance therapy seems to be still inadequate. The results of trials with more vigorous regimes are awaited. The complications of therapy still remain to be solved. Apart from the well-known complications wuch as hepatitis, haemolytic disease and F VIII inhibitors, the existence of previously unnoticed complications as splenomegaly, hypertension, renal disease and paradoxal bleeding have been recently realized. The role of altered fibrinogen, fibrin degradation products (FDP) and unclassified fibrinogen derivatives (UFD) present in cryoprecipitates and F VIII concentrates in the above complications needs to be further clarified. In conclusion, tremendous progress in various aspects of hemophilia has been achieved in developed countries. Comprehensive care can now be carried out in various centers. On the other hand, developing countries still face a number of basic problems. The concept that hemophilia is a "manageable" disease and that chronic crippling and death from exsanguination can be prevented, should be disseminated widely by various means...

Antifibrinolytic Agents↗

PubMed1979

[A new treatment method for hydrochloric acid burns of the hand].

The progression of hydrofluoric acid corrosion on the hands, which cannot be arrested by other means and leads to crippling or amputation, can be stopped by one single or several repeated intra-arterial perfusions of 10 ml 20% calcium-gluconate solution in 40 ml normal saline over 4 hours as close to the defect as possible via the brachial artery or one of the forearm arteries. In 4 patients, with corrosion of a severity judged to be between first and third degree, spontaneous healing resulted after this treatment. Based on our experience we advise the use of intra-arterial perfusion and show its advantages over subcutaneous injections or intravenous infusion of calcium.

Adult↗

PubMed1969

Current concepts of hyperuricemia and gout.

Recent studies have confirmed that gout is an inborn error of metabolism. It has now become evident that the hyperuricemia associated with gout might occur either due to overproduction of uric acid, underexcretion of uric acid or a combination of these processes. Furthermore, patients with excessive purine synthesis may have a specific enzyme defect resulting in altered feedback inhibition of purine synthesis. A neurological disease manifest by mental retardation, choreo-athetosis, aggressive behavior, lip-biting and self-mutilation and associated with decidedly increased purine biosynthesis serves as a prototype of this kind of disorder. Other defects in regulation of purine biosynthesis have been postulated but their existence not yet confirmed. It has been demonstrated that urate crystals which are deposited from hyperuricemic body fluids set up an acute inflammatory reaction by means of a variety of chemical mediators. Thus, acute gouty arthritis is now recognized as an example of "crystal induced" synovitis. The treatment of gout consists of (1) the control of acute gouty attacks, and (2) the maintenance of normal serum uric acid concentrations. This latter may be achieved either with uricosuric drugs or with xanthine oxidase inhibition. With these principles in mind, it is now possible to avoid many of the severe crippling effects of gout and to restore the vast majority of gouty patients to useful and productive lives.

Chemical Phenomena↗

PubMed1965

Rehabilitation of the aged.

The rehabilitation needs and goals of older people differ in many respects from those of the young. In younger individuals a crippling condition may affect an otherwise healthy body, while in an older person it may be superimposed on other pre-existing degenerative diseases. Thus, in older patients the restorative or rehabilitative phase rarely can be separated from the phase of definitive medical treatment.The primary goal of rehabilitation of younger individuals is usually vocational. In the older group this goal or objective is, by and large, secondary. This does not minimize, however, the value of medical and social services in the rehabilitation of older persons. The simple ability to care for his own personal needs can do much to help the elderly disabled patient regain his dignity and self-respect and remove his fears of becoming a burden on his family or society.

Aged↗

PubMed1966

Revascularization of the entire heart by internal mammary artery implantation, epicardiectomy and free omental graft.

The indications for and the contraindications to total cardiac revascularization are described on the basis of the author's experience. The combined operation of internal mammary artery implant, epicardiectomy and free omental graft was performed on 62 patients. Of 45 who did not have angina at rest without exciting cause (Grade I) 39 showed improvement; 32 returned to full-time work, 18 of whom had been unable to work before operation. There were two operative deaths. Among the 17 patients who were "bed-chair cripples" (angina at rest without cause-Grade II), there was a 24% operative mortality (four deaths), but 76% of the survivors had marked improvement. Only one of this group had been working full-time preoperatively; now nine are working full-time.

Angina Pectoris↗

PubMed1966

Hemorrhage during long-term anticoagulant drug therapy. 1. Intracranial hemmorrhage.

Intracranial hemorrhage was the most serious hemorrhage as measured by death and disability, occurring during long-term anticoagulant drug therapy of 1,626 patients. Among 95 hemorrhagic episodes considered life-threatening or potentially crippling, 30 were intracranial and 56 were gastrointestinal. Over two-thirds of the patients with intracranial hemorrhage died, as against one-tenth of those with gastrointestinal hemorrhage. The incidence of intracranial hemorrhage is increased among hypertensive patients, but the results of a controlled study indicate that the incidence of intracranial hemorrhage is not affected by whether or not the hypertensive patient is receiving anticoagulant therapy. Hypertension is the important precipitating factor, not the prothrombin level. Even at excessively low prothrombin levels only one intracranial hemorrhage occurred in 337 instances. In this series, reducing coagulability to a desirable range did not increase the probability of intracranial hemorrhage. Once bleeding occurred, however, it increased the risk of death and disability.

Anticoagulants↗

PubMed1977

The external compression syndrome of the ulnar nerve at the cubital tunnel.

Diagnosis of the cubital tunnel external compression syndrome, and subsequent avoidance of further external pressure, minimizes the possibility of progressive crippling of the hand. The usual clinical features are local tenderness over the cubital tunnel, often accompanied by distal paresthesias, and neurological deficit in the ulnar nerve distribution with sparing of the flexor digitorum profundus and flexor carpi ulnaris muscles; the elbow flexion test, described by the author, awaits evaluation in the diagnosis of the syndrome. Clinicians and others concerned with positioning patients on the operating room table or caring for patients in the ward should be aware of the syndrome. Avoidance of a position of the elbow which predisposes to external compression of the cubital tunnel is mandatory and active elbow movement should be encouraged in bedridden and chair-bound patients. Surgical treatment is sometimes indicated, at least to halt progression of the palsy. A classification of the cubital tunnel syndrome is proposed: physiological, acute and subacute due to external pressure (both forming the cubital tunnel external compression syndrome) and chronic (space-occupying lesions and loss of volume due to lateral shift of the ulnar as a consequence of childhood injury to the capitular epiphysis). Nerve conduction studies may be helpful in the diagnosis of the doubtful cubital tunnel syndrome, particularly when there is definite impairment of power or sensation in the hand.

Acute Disease↗