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PubMed1985

Myoelectric training methods for the preschool child with congenital below-elbow amputation. A comparison of two training programmes.

The Ontario Crippled Children's Centre has completed a two-year research project designed to develop effective strategies for training the preschool child to use a myoelectric prosthesis. Two programmes were developed: one home-based with the parent as primary trainer, and the other Centre-based with a therapist as trainer. Seventeen children were successfully trained and fitted with myoelectric prostheses. Both training programmes appear to be equally effective, proving that informed parents can assume responsibility for the training of their children. Economic implications are self-evident. The functional assessment of the children's skill with the myoelectric prosthesis is very encouraging so far. However, long-term studies are indicated for adequate assessment of the cost-effectiveness of early myoelectric fitting. As a result of this study, effective training methods can now be used in routine clinical service; a manual is available to provide guidelines.

Age Factors↗
PubMed1985

Multiple epiphysial dysplasia in one family. A further review of seven generations.

A family of 114 people in six generations, of whom 45 were affected by multiple epiphysial dysplasia, was described in this Journal in May 1960. We have been able to review them again 23 years later, and also to report on the type and incidence of the disease in more children and in a further generation. The relative severity of the disease in different branches of the family has been unchanged through the generations. Patients who were previously hopelessly crippled can today be rehabilitated by total hip replacement. Genetic counselling and birth control have reduced the relative and absolute numbers of affected children; this is tending to make the disease self-limiting.

Adolescent↗
PubMed1988

Neonatal screening for congenital dislocation of the hip. A prospective 21-year survey.

A prospective neonatal screening programme for congenital dislocation of the hip is reported. This covered over 20,000 live births including all the babies born in one area, with a virtually complete follow-up. All babies were examined within the first seven days of life and all hips which were dislocated or unstable after four days (1.6%) were splinted. Only two screened cases were "missed" and subsequently detected at 15 and 18 months. No later cases were discovered, and patients between 10 and 20 years of age had apparently remained asymptomatic. The detailed results show that neonatal screening is effective in detecting and preventing a crippling disorder in otherwise normal people. The examination, though brief, is difficult to perform well; it is considered that failure of the method lies with the examiner and not with the tests.

Female↗
PubMed2003

Hyperbaric oxygen therapy as a treatment for stage-I avascular necrosis of the femoral head.

Avascular necrosis (AVN) of the head of the femur is a potentially crippling disease which mainly affects young adults. Although treatment by exposure to hyperbaric oxygen (HBO) is reported as being beneficial, there has been no study of its use in treated compared with untreated patients. We selected 12 patients who suffered from Steinberg stage-I AVN of the head of the femur (four bilateral) whose lesions were 4 mm or more thick and/or 12.5 mm or more long on MRI. Daily HBO therapy was given for 100 days to each patient. All smaller stage-I lesions and more advanced stages of AVN were excluded. These size criteria were chosen in order to compare outcomes with an identical size of lesion in an untreated group described earlier. Overall, 81% of patients who received HBO therapy showed a return to normal on MRI as compared with 17% in the untreated group. We therefore conclude that hyperbaric oxygen is effective in the treatment of stage-I AVN of the head of the femur.

Adult↗
PubMed1999

Desensitization to allopurinol in a patient with previous failed desensitization.

OBJECTIVE: To describe an allopurinol desensitization that failed on the first attempt but was successful on the second attempt, resulting in the management of crippling tophaceous gout. CASE SUMMARY: A 64-year-old white man with a history of gouty nephropathy requiring hemodialysis developed a severe cutaneous reaction from exposure to allopurinol. The first desensitization attempt was unsuccessful, and the gouty nephropathy caused chronic cellulitus and urate microcrystal deposition on the patient's hands and feet. Continuous ambulatory peritoneal dialysis and hemodialysis were used to treat the patient's severe symptoms and increase uric acid clearance. This method, however, was ineffective. Severe tissue ischemia, requiring bilateral below-the-knee amputations, prompted the second desensitization attempt four years later. The second attempt, administered differently and with more caution, was successful and did not cause further complications. DISCUSSION: Allopurinol is the only agent available to effectively reduce uric acid concentrations in those who are over-producers. Hypersensitivity-type reactions have been reported with its use, and desensitization is the only viable therapeutic option. Few cases of desensitization to allopurinol have been reported in the literature. This article describes a failure and a subsequent success in desensitization to allopurinol. CONCLUSIONS: Although desensitization to allopurinol poses potential risks, the benefits can outweigh the risks of therapy. Desensitization requires close monitoring; if failure does occur, subsequent attempts can be successful, as this case report demonstrates.

Allopurinol↗
PubMedDate not supplied

A conservative case for universal access to health care.

Universal access to health care has historically faced strident opposition from political conservatives in the United States, although it has long been accepted by most conservatives in the rest of the industrialized world. Now, in a global economy where American business is crippled by the rising cost of market-based health care, the time may be ripe for change. The key to fostering a new mindset among American conservatives is to show why universal access fulfills many of the basic values that all conservatives hold.

Health Care Sector↗
PubMed1990

The great empty cup of attention: the doctor and the illness in the Wings of the Dove.

James confers on his characters his deepest fears and truths. He incorporates within his writing both his stunning and at times crippling intellectual powers and his dark inchoate mournful passion and remorse. The very events that made him a casualty are the experiences he grants to his characters to endure, to grapple with, and to master. In Wings James settles with his characters and with his readers the effects of these two deaths in his life. He was flattened by Minny's and Fenimore's deaths. He, like Marcher, cannot grasp the extent of his paralysis. It dawns on him like the sadness of a winter dawn but also like the explosive epiphany of Marcher at the grave that he has not lived. The words of Howell quoted by Sturges, the events of Wings, The Ambassadors, "The Beast in the Jungle" all reinforce what he knows to be true. He has not lived, he has not had what that man had to make him, by the loss of it, so bleed and yet live. What, after all, is more important in any life than the ability to love and the capacity to face death? This is the heart of the matter that James takes on in Wings. In the structure of the novel, to become conscious is to be in danger. Milly, the patient, achieves what eludes the other characters. Kate, Maud, Densher, Susan all are unable to raise their heads above their own lives. Only Milly is aware of herself within and beyond the book. Perhaps Densher begins in the very end to raise his head similarly, and in the sequel of "Beast," he achieves it.(ABSTRACT TRUNCATED AT 250 WORDS)

Attitude to Health↗
PubMed2002

The Role of Pancreatic Duodenum Homeobox Protein-1 in the Development of Diabetes Mellitus.

Pancreatic duodenum homeobox protein-1 (Pdx-1, also known as Idx-1, Ipf-1, Stf-1) is a homeodomain protein of 283 amino acids whose expression is required for pancreas development to occur. This protein is now recognized to be a critically important transcription factor involved in the regulation of the expression of multiple genes essential for the proper functioning of the beta cells in the endocrine pancreas (islets of Langerhans) that produce insulin, a lack of which results in the development of diabetes mellitus. Finding a way to increase Pdx-1 expression and the neogenesis of beta cells, perhaps through the stimulating effects of glucagon-like peptide-1, may be useful for the treatment of this crippling disease. (c) 2002 Prous Science. All rights reserved.

Journal Article↗
PubMed2003

Hypoglycemic effect of Hypoxis hemerocallidea corm (African potato) aqueous extract in rats.

Diabetes mellitus has been recognized as a clinical syndrome since ancient times, and remains a crippling global health problem today. It is a group of heterogeneous, autoimmune, hormonal and metabolic disorders, often accompanied by hypertension, hyperlipidemia and obesity. Current estimates suggest that approximately 150 million people worldwide suffer from diabetes mellitus. The present study was undertaken to examine the hypoglycemic effect of aqueous extract of Hypoxis hemerocallidea (family: Hypoxidaceae) corm (locally known as "African Potato") in normal (normoglycemic) and in streptozotocin (STZ)-treated, diabetic rats. Young adult, male Wistar rats weighing 250-300 g were used. Diabetes mellitus was induced in the group of diabetic test rats by intraperitoneal injections of STZ (90 mg/kg). In one set of experiments, graded doses of the aqueous extract of African Potato (100-800 mg/kg p.o.) were administered to 12-h fasted normal and diabetic rats. In another set of experiments, 800 mg/kg of African potato extract, a dose of the plant extract that produced maximal hypoglycemic effects in fasted normal and diabetic rats in our pilot experiments, was used. The hypoglycemic effect of this single dose was compared with those of insulin (5 micro U/kg s.c.) and glibenclamide (5 mg/kg p.o.) in 12-h fasted normal and diabetic rats. Following acute treatment, relatively moderate to high doses of African potato extract (100-800 mg/kg p.o.) produced dose-dependent, significant reductions (p < 0.05-0.001) in the blood glucose concentrations of fasted normal and diabetic rats. Similarly, insulin (5 micro U/kg s. c.) and glibenclamide (5 mg/kg p.o.) produced significant reductions (p < 0.01-0.001) in the blood glucose concentrations of the fasted normal and diabetic rats. At a dose of 800 mg/kg, the plant extract caused 30.20% and 48.54% reductions in the blood glucose concentrations of fasted normal and STZ-treated diabetic rats, respectively. While it is likely that the hypoglycemic effect of the plant extract is largely due to its phytosterols and/or sterolin content, the exact mechanism of its hypoglycemic action is still obscure and will have to await further studies. However, the results of this experimental animal study indicate that African potato possesses hypoglycemic activity; and thus lends credence to the suggested folkloric use of the herb in the control and/or management of adult-onset, type 2 diabetes mellitus in some communities of South Africa.

Administration, Oral↗
PubMed2007

Recovery from skeletal fluorosis (an enigmatic, American case).

UNLABELLED: A 52-year-old man presented with severe neck immobility and radiographic osteosclerosis. Elevated fluoride levels in serum, urine, and iliac crest bone revealed skeletal fluorosis. Nearly a decade of detailed follow-up documented considerable correction of the disorder after removal of the putative source of fluoride (toothpaste). INTRODUCTION: Skeletal fluorosis, a crippling bone disorder, is rare in the United States, but affects millions worldwide. There are no data regarding its reversibility. MATERIALS AND METHODS: A white man presented in 1996 with neck immobility and worsening joint pains of 7-year duration. Radiographs revealed axial osteosclerosis. Bone markers were distinctly elevated. DXA of lumbar spine (LS), femoral neck (FN), and distal one-third radius showed Z scores of +14.3, +6.6, and -0.6, respectively. Transiliac crest biopsy revealed cancellous volume 4.5 times the reference mean, cortical width 3.2 times the reference mean, osteoid thickness 25 times the reference mean, and wide and diffuse tetracycline uptake documenting osteomalacia. Fluoride (F) was elevated in serum (0.34 and 0.29 mg/liter [reference range: <0.20]), urine (26 mg/liter [reference range: 0.2-1.1 mg/liter]), and iliac crest (1.8% [reference range: <0.1%]). Tap and bottled water were negative for F. Surreptitious ingestion of toothpaste was the most plausible F source. RESULTS: Monitoring for a decade showed that within 3 months of removal of F toothpaste, urine F dropped from 26 to 16 mg/liter (reference range: 0.2-1.1 mg/liter), to 3.9 at 14 months, and was normal (1.2 mg/liter) after 9 years. Serum F normalized within 8 months. Markers corrected by 14 months. Serum creatinine increased gradually from 1.0 (1997) to 1.3 mg/dl (2006; reference range: 0.5-1.4 mg/dl). Radiographs, after 9 years, showed decreased sclerosis of trabeculae and some decrease of sacrospinous ligament ossification. DXA, after 9 years, revealed 23.6% and 15.1% reduction in LS and FN BMD with Z scores of +9.3 and +4.8, respectively. Iliac crest, after 8.5 years, had normal osteoid surface and thickness with distinct double labels. Bone F, after 8.5 years, was 1.15% (reference range, <0.1), which was a 36% reduction (still 10 times the reference value). All arthralgias resolved within 2 years, and he never fractured, but new-onset nephrolithiasis occurred within 9 months and became a chronic problem. CONCLUSIONS: With removal of F exposure, skeletal fluorosis is reversible, but likely impacts for decades. Patients should be monitored for impending nephrolithiasis.

Biopsy↗
PubMed1999

Alpha1-adrenergic hypothesis for pulmonary hypertension.

Pulmonary hypertension (PH) is a chronic and disabling condition that affects the pulmonary vasculature. Once PH is diagnosed, the prognosis is generally poor with a rapid downhill course. PH management is largely empirical because the underlying pathophysiologic mechanisms that are responsible for the excessive vasoconstrictor and vascular smooth muscle proliferative responses are poorly understood. Based on new information concerning the role of adrenergic receptors in regulating various cellular functions, a new perspective on the genesis of PH has emerged, along with a unifying hypothesis for the role of alpha1-adrenergic receptors present in the pulmonary vasculature as the major contributor to the pathophysiologic changes associated with PH. Adrenergic receptors that are present on vascular smooth muscle cells regulate vascular tone and growth. The alpha1-adrenergic receptors that are present on the small- and medium-sized pulmonary arteries have a unique and greatly enhanced affinity and activity to alpha1-adrenergic agonists. Under physiologic conditions, this helps in regulating vascular tone and maintains an adequate ventilation/perfusion matching. However, the excessive stimulation of alpha1-adrenergic receptors produces not only smooth muscle contraction but also proliferation and growth. The conditions that produce an increase in alpha1-adrenoreceptor gene synthesis, density, and activity (such as hypoxia or changes in vessel wall pressure) or increase the levels of its agonists (such as norepinephrine, appetite suppressants, or cocaine) greatly enhance pulmonary vascular smooth muscle contractile and proliferative responses and lead to the development of PH. An understanding of the role played by these receptors in the pathophysiology of PH would not only help to avoid the use of alpha1-agonists for appetite suppression and other disease states, but also would help in developing new drugs to block these receptors. A further understanding of the alpha1-adrenoreceptor subtypes present in the pulmonary vasculature, the factors that regulate their expression, and their intracellular signaling pathways would help researchers to devise newer therapeutic strategies and, hopefully, to find a cure for this crippling condition.

Animals↗
PubMed2005

Impact of environment upon gender identity and sexual orientation: a lesson for parents of children with intersex or gender confusion.

Two histories of physically normal men with persistent gender issues highlight the major impact played by parental input on the sexual and gender development of children. Both men had been subjected to firm, harsh behavior modification by their parents, particularly their mothers, during childhood in response to effeminate behavior. While both men continue to manifest major gender/sexual issues as adults, their outcomes have been dramatically different. The first man takes female hormones and denies any satisfaction from his sexuality. This individual remains convinced that he has female internal sexual organs and monthly internal menstrual bleeding. Although he has a career, he has become alienated from his family and is a social cripple. The second man has a successful career, lives a heterosexual life with his children and wife of 20 years and is involved in his community. He is visually attracted to men and remains obsessed with male pornography. This individual credits his mother with directing him toward a successful heterosexual life, which he feels has prevented the emotional burden of an active homosexual life. These two cases illustrate the tremendous influence played by environment and parental input on the sexual perspectives of developing children.

Adaptation, Psychological↗
PubMed2006

Surgical, medical and psychological dilemmas of sex reassignment: report of a 46,XY patient assigned female at birth.

This is a report of a 16 year-old 46,XY male who was reassigned female and had feminizing surgery during infancy because of what was judged to be inadequate genital masculinization. This patient had a dysgenetic testis that was shown to be producing testosterone during infancy. Although initially the reassignment appeared to be successful, psychological problems became progressively more severe during childhood to incapacitation by age 10 years. After it was verified that he had a male sexual identity, reassignment as male began, initially by living as a boy, then with testosterone therapy. Staged phalloplasty surgery was begun at age 16 years. Currently he has an adult-sized penis, although its function is not yet clear. Sadly, none of the steps to align his sex assignment to his perception as male has significantly alleviated his psychological issues and he continues to be severely impaired and socially compromised. Major issues include the crippling psychiatric disease that is resistant to psychotherapy and surgical problems with phalloplasty after surgery at infancy that involved reduction of the phallus with recession of the glans to the typical clitoral location. The glans was left intact at the anterior base of the phallus. Genital responsiveness during sexual activity and satisfaction are as yet unknown.

Adaptation, Psychological↗
PubMed2006

A multimodality approach to the treatment of craniopharyngiomas avoiding hypothalamic morbidity: a UK perspective.

The management of craniopharyngiomas is complex and controversial. The perception that they are benign tumours cured by radical surgical resection is not borne out by their often difficult excision, propensity to recur and invade, and high late morbidity and mortality from direct brain injury. Their central location makes visual or pituitary dysfunction and/or hydrocephalus common presenting features. The most important consequence of craniopharyngiomas is hypothalamic injury which may result in severe, crippling and life-threatening sequelae, such as adipsia, morbid obesity, sleep, and behavioural and cognitive disorders. Evidence suggests that unless the tumour is smaller than 2-4 cm in the mid-line and completely resectable without additional hypothalamic or visual compromise, the surgical approach should be conservative even if this leaves residual tumour. Adjuvant focal radiotherapy to residual or recurrent disease has proven efficacy in long-term tumour control. Thus concerns regarding the potential late toxicity of radiotherapy to the developing brain need to be balanced against the recognised morbidity and mortality of recurrent tumour and repeated neurosurgical interventions. Hypothalamic damage is in many cases surgically induced. Given the devastating consequences of such an injury this should be avoided at all costs. Thus the aims of primary treatment should be to relieve tumour-associated compression symptoms, preserve (or improve) vision and hypothalamo-pituitary function, and minimise tumour recurrence with its attendant high morbidity and mortality. National registration and management by multidisciplinary teams in specialised centres according to nationally agreed risk adapted treatment strategies are likely to improve outcomes, as has been demonstrated for example in adult pituitary tumours. Because of the rarity of craniopharyngiomas, international collaborative trials are necessary to properly inform future therapies.

Combined Modality Therapy↗
PubMed2000

The parathyroid hormone, its fragments and analogues--potent bone-builders for treating osteoporosis.

As populations age a rising number of men and women, but especially women during the first decade after menopause, become victims of a severe, accelerated loss of bone with crippling fractures known as osteoporosis. This often results in costly, prolonged hospitalisation and perhaps indirectly, death. Osteoporosis in women is caused by the menopausal oestrogen decline, which removes several key restraints on the generation, longevity and activity of bone-resorbing osteoclasts. Although there are many antiresorptive drugs on or coming onto the market (calcitonin, bisphosphonates, oestrogen and SERMS) that can slow or stop further bone loss, there are none that can restore lost bone mechanical strength by directly stimulating osteoblast activity and bone growth. However, there is a family of potent bone-building peptides, namely the 84 amino acid parathyroid hormone (PTH). Its 31 to 38 amino acid N-terminal fragments are currently in or about to enter clinical trials. We can predict that these peptides will be effective therapeutics for osteoporosis especially when supplemented with bisphosphonates or SERMs to protect the new bone from osteoclasts. These peptides should also accelerate the healing of fractures in persons of all ages and restore lost bone mass and mechanical strength to astronauts following their return to earth after long voyages in space.

Animals↗
PubMed2003

Etanercept in psoriatic arthritis.

Psoriatic arthritis (PsA), an inflammatory arthritis associated with psoriasis, can lead to disability from progressive joint destruction and bony fusion. To date, conventional disease modifying antirheumatic drugs (DMARDS) have not convincingly lessened joint pain and inflammation in PsA and there is very little data on the limitation of radiographic progression with these agents. The biological agent etanercept (Enbrel, Amgen, Inc, Thousand Oaks, California, USA) is a soluble TNF receptor fusion protein with proven efficacy in the treatment of rheumatoid arthritis (RA). In a Phase II and Phase III trial, conducted in moderate-to-severe PsA, etanercept significantly reduced joint pain and swelling and lowered the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level. A significant decline in structural damage was observed as early as 6 months after starting the drug. Etanercept also improved quality of life measures (Health Assesment Questionnaire [HAQ] and global assessment scores). Up to a third of patients experienced transient injection-site reactions. Rare cases of opportunistic infection, demyelinating disorders and aplastic anaemia have been reported, but a causal link has not been established. In summary, etanercept is a safe and effective agent for the treatment of PsA and represents a major advance in the therapy of this potentially crippling disease.

Animals↗
PubMed2005

Therapy for haemophilia: recent advances and goals for the future.

In the 20th century, haemophilia evolved from a life-threatening, crippling disease to one for which the prognosis is excellent and many patients lead normal, productive lives. Although dramatic achievements in the treatment of haemophilia have occurred, the current therapies have significant drawbacks. Among these is the relatively high incidence of inhibitor development, the requirement for frequent intravenous infusions to prevent bleeding complications, the lack of effective treatment for established joint disease, and the high cost of treatment. The future goal of haemophilia treatment first and foremost is curing this genetic condition via gene therapy. As this goal is likely many years away, improvements in the current factor products in order to reduce the development of inhibitors and to reduce the frequency of therapy are more immediately achievable goals. Finally, improving the treatment of bleeding complications, particularly in inhibitor patients, and developing novel adjunctive therapies for the management of joint disease are also important goals for the near future. This review will discuss in detail the cu-rrent and future goals of haemophilia therapy.

Clinical Trials as Topic↗
PubMed1991

Freud's antipathy to America.

Freud first saw this in America's attitude toward the use of cocaine. It was freely and legally used and even promoted by American physicians as beneficial in many medical conditions. Freud was first swept along by America's prococaine enthusiasm. He narrowly missed destroying his own medical reputation by his continuing close connection and advocacy of cocaine. Freud made his only visit to America in 1909. Despite the success of his visit and the congeniality of Americans toward him, he was already programmed toward a negative response. He allowed minor everyday inconveniences and cultural differences to spoil the trip for him. The chronic intestinal distress that had bothered him previously now was labelled as his "American dyspepsia." He developed additional reasons for disliking America. They accepted his three key dissenters, Adler, Jung, and Rank. He even believed that these three men achieved greater notoriety and popularity in America than he did. In fact, the Freudian psychoanalytic movement became much larger and more powerful in America than did the followings of any or all of his defectors. Freud did not like the shortcuts and lack of mastery of the basics of psychoanalysis that he believed happened in America. Some of this was true, but it was mainly exaggerated in Freud's mind. The gold of psychoanalysis was never transformed into a practical but deficient psychoanalytic alloy as Freud feared it might. There was a genuine disagreement about whether a psychoanalyst should have to become a medical doctor or not. It was a complicated issue in America because of laws that said that doing psychoanalysis was practicing medicine. Freud viewed the American stand against recognizing lay analysts as more evidence of American's being a rebellious son. Economic factors also played a significant factor in Freud's dislike of America. Freud was initially enthusiastic about President Wilson and his plans for peace. He found after World War I that all of his savings were wiped out and that Vienna suffered from a crippling inflation. As a result of this he had to take increasingly more American analysands as they paid him in valuable American dollars. He resented this financial dependency on Americans. He also had difficulty communicating with these American analysands and largely blamed them for their undisciplined way of speaking. What I have presented is a survey or overview of the reasons for Freud's dislike of America.(ABSTRACT TRUNCATED AT 400 WORDS)

Austria↗