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At least 829 records · Page 46Linked to original sources
PubMed1985

Childhood sarcoidosis in Arkansas.

Sarcoidosis, a granulomatous disease of unknown etiology, is most often seen in young adults. Childhood cases have been reported primarily from Virginia, North Carolina, and South Carolina. Thirty children have been seen in Arkansas between 1957 and 1982, which suggests that the endemic area for childhood sarcoidosis should include both the south central and southeastern United States. The median age was 11 years; 29/30 were black; and 90% were symptomatic, 60% with systemic symptoms. Manifestations included abnormal chest roentgenograms (100%), restrictive pulmonary functions (79%), lymphadenopathy (63%), splenomegaly (40%), skin lesions (30%), granulomatous uveitis (27%), hyperglobulinemia (72%), and hypercalcemia (30%). Course and prognosis were similar to those in adults at follow-up of two to 11 years. Four with uveitis had serious residua in the eyes, three had crippling restrictive lung disease, and two died of respiratory failure. Sarcoidosis seems to be an immunologic response to an unknown inhaled antigen, probably present in the southeastern and south central US.

Adolescent↗
PubMed1996

Pseudo-multiple sclerosis.

Multiple sclerosis (MS) is among the most crippling diseases of the nervous system in young adults, and diagnosis can be difficult. It is true that MS can exist without magnetic resonance imaging or cerebrospinal fluid abnormalities, but whenever there are an atypical clinical pattern and no confirmatory laboratory tests, then "pseudo-MS" might be considered. Six cases of presumed pseudo-MS are reported. Psychologic treatment of these cases was unsatisfactory. Treatment in all such cases will be difficult, but more so when MS cannot be disproved and when the patient resists a psychogenic explanation for the symptoms.

Adult↗
PubMed1997

Back pain in children and adolescents: a retrospective review of 648 patients.

Back pain in children has long been considered a serious condition with an organic cause. The staff of the Shriner's Hospital had been informally noting that diagnoses among children with back pain had been changing. We conducted a retrospective study of 648 patients with spinal disorders treated at the Shriner's Hospital for Crippled Children, Spokane Unit, between January 1991 and June 1993. The patients were divided into two groups: those with pain and those without pain. It was found that back pain with no organic cause was the primary diagnosis in 57% of the group having pain. Only one child had a diagnosis of malignancy, and there were no cases of active infection. In the group with pain, three interesting associations were found concerning psychosocial problems, disability, and litigation. It was found that the diagnoses in children with back pain treated at this referral center during the study period parallel those in the adult population with back pain.

Adolescent↗
PubMed1989

Social support and psychological adjustment to chronic coronary heart disease: operationalization of Johnson's behavioral system model.

No comprehensive theory of social support and psychological adjustment to chronic illness such as coronary heart disease (CHD) exists. This article reviews the literature on social support and adjustment to CHD from the perspective of Johnson's behavioral system model. It is argued that the quality of social support or nurturing is the major factor predicting cardiac crippled behaviors or dependency following myocardial infarction (MI). The variables of self-esteem, anxiety, depression, and perceptions of functional capacity are identified as variables affecting choices such as return to work and adherence to the treatment regimen and the behavioral outcome of dependency following MI.

Adaptation, Psychological↗
PubMed2000

Primary non-small-cell lung cancer: determining the suitability of the patient and tumor for resection.

Choosing resective surgery for patients with bronchogenic carcinoma requires assessments of tumor suitability and patient suitability. Tumor suitability is largely dependent on the assessed stage of the tumor complex, based on characteristics of the primary tumor, detection of lymph node metastases, and detection of distant metastases. Imaging tests that assist in the determination of tumor stage include computed tomographic scans and positron emission tomographic (PET) scans. PET scans are more sensitive and specific than computed tomography. PET is also helpful in screening for distant metastases. Mediastinoscopy is required in most cases of mediastinal adenopathy. Patient suitability is assessed by predicting short-term surgical mortality, and the likelihood of crippling long-term respiratory failure. There is no single test that provides such information. Pulmonary function tests can be used to calculate the "predicted postoperative" function, and several algorithmic approaches have been devised to predict surgical risk. Assessments of regional pulmonary function are obtained with quantitative perfusion scintiscans. Cardiac function is also an important factor.

Carcinoma, Non-Small-Cell Lung↗
PubMed2001

Cell surface fucose ablation as a therapeutic strategy for malignant neoplasms.

The sugar alpha-L-fucose is overexpressed in many human malignancies, especially on specific glycoproteins, glycolipids, certain mucins, and putative cell adhesion ligands found on cancer cell surfaces. Many of these molecules are known or suspected mediators of cell-cell adhesion, cell signaling, motility, or invasion. As knowledge of fucose metabolism evolves and specific mechanisms of its distribution and incorporation are more exactly documented, modulation of fucose expression in cancer is becoming increasingly more feasible. The authors propose that cancer cell surface alpha-L-fucose is a logical target for selective therapeutic ablation. Reduction of fucose content on the surfaces of malignant cells should effectively cripple the cells' physiologic functions by altering or dysregulating cell-cell or cell-matrix interactions, critical for maintaining the malignant phenotype. Significant therapeutic benefits might include modulation of adhesion abnormalities in the cancer cells, reduction of cancer cell motility or invasiveness, reexposure to immune surveillance, or a combination of these events.

Antigens, Surface↗
PubMed2002

Unexpected complication of posterior canal occlusion surgery for benign paroxysmal positional vertigo.

OBJECTIVE: The purpose of this report was to illustrate how an unusual complication of posterior canal occlusion surgery for benign paroxysmal positional vertigo (BPPV) may be recognized and prevented. Recurrence of BPPV after occlusion surgery of the posterior semicircular canal has not previously been reported in the literature, to the authors' knowledge. Failure of occlusion not only permits the continued symptoms of BPPV but also burdens the patient with the additional morbidity of a fistula of the PSCC. The authors describe the successful treatment of a patient with recurrent and incapacitating BPPV after the failure of occlusion surgery of the posterior semicircular canal, when the patient was simultaneously crippled by the distressing morbidity of an iatrogenic fistula. DESIGN: Case report. SETTING: Tertiary care referral center. INTERVENTION: Surgical excision of the fistulous segment of the membranous posterior semicircular canal. OUTCOME MEASURES: The BPPV was resolved. The distressing symptom of a fistula was eliminated. Hearing was preserved. CONCLUSION: Failure to completely occlude the posterior semicircular canal during posterior canal occlusion surgery results in recurrence of BPPV and an iatrogenic fistula, both of which are preventable. The occurrence of such an event is described, its management is outlined, and some thoughts are offered about its prevention.

Electronystagmography↗
PubMedDate not supplied

Use of Joshi External Stabilizing System in postburn contractures of the hand and wrist: a 20-year experience.

Postburn contractures of the hand and wrist can range from a minor cosmetic problem to a crippling condition. The contractures, initially limited to the skin, extend to all the soft-tissue structures over time, often necessitating capsulotomies and tendon lengthening, although they still may not be amenable to total correction. The Joshi External Stabilizing System (JESS) is a versatile, lightweight external fixator consisting of K wires, distractors, and connecting rods (both hinged and nonhinged) along with various link joints. JESS is a dynamic system that allows the lengthening of the contracted tissues via slow distraction, causing minimal surgical insult. This is a retrospective review of 218 cases of postburn contractures of the hand treated with JESS during the last 20 years. Deformities varied from finger contractures to metacarpophalangeal joint and wrist contractures. All cases were of long duration at the time of presentation with the original injury being 4 months to 20 years old. All were assessed for the degree of function and deformity. The patients' activities of daily living were recorded and x-rays taken to evaluate joint configuration. The patients underwent a conservative surgical release followed by application of the appropriate JESS frame for the correction of the residual deformity. After 6 weeks, the frame was removed and hand therapy continued. An analysis is provided of the outcome. The technique produces functional hands with minimal surgical insult.

Adolescent↗
PubMed2003

Radiation increases fibrogenic cytokine expression by Peyronie's disease fibroblasts.

PURPOSE: Peyronie's disease is a crippling penile deformity that results from fibrosis in the tunica albuginea. To our knowledge its cause is unknown and empirical therapies are used extensively. A factor involved in the development of Peyronie's disease is fibrogenic cytokine over expression. Radiation therapy is an empirical therapy for this condition and, while some data suggest a role for it, no literature exists on the effects of radiation on tunical tissue or cells derived from this tissue. We evaluated the effect of radiation on fibrogenic cytokine production in cells cultured from Peyronie's disease plaque tissue. METHODS AND MATERIALS: Using a well established cell culture model cells derived from Peyronie's disease plaque tissue and neonatal foreskins were irradiated with 5 Gy (treatment group) or left nonirradiated (control group). At 24 hours cells were harvested and the supernatant was analyzed using enzyme-linked immunosorbent assay to determine the levels of the 2 fibrogenic cytokines basic fibroblast growth factor and platelet-derived growth factor-AB. RESULTS: Four Peyronie's disease plaque derived cultures and 2 neonatal foreskin derived cultures were analyzed. All plaque derived fibroblasts demonstrated significant elevations in basic fibroblast growth factor and platelet-derived growth factor-AB compared with foreskin derived fibroblasts. CONCLUSIONS: These data suggest that radiation may in fact increase the production of fibrogenic cytokines, which may promote the fibrotic process involved in Peyronie's disease. Further study is aimed at defining the effect of irradiation on plaque tissue.

Cells, Cultured↗
PubMed2005

Management and outcome of complex hypospadias repairs.

PURPOSE: Repeated attempts at surgical repair of hypospadias may leave the penis scarred, hypovascular and shortened. We report on the clinical outcomes of our repaired complex hypospadias cases, often referred to as hypospadias cripples. MATERIALS AND METHODS: We evaluated the records of 137 children and young adults from January 1980 through December 2002 who were referred to us after multiple unsuccessful hypospadias repairs. The records of 11 patients were inadequate. The ages of the remaining 126 patients ranged from 14 months to 35 years. The number of prior surgical procedures ranged from 2 to 23. Of the 126 cases 98 (78%) were repaired in a single stage (group 1) and 28 (22%) underwent multistage repairs (group 2). RESULTS: Major complications occurred in 17 cases (17%) in group 1 and in 2 (7%) in group 2. Major complications included repair breakdown, stricture, diverticulum and multiple fistulas. Minor complications occurred in 9 (9%) cases in group 1 and in 4 (14%) in group 2. Minor complications included a single small urethrocutaneous fistula, skin tethering, inclusion cysts and glandular irregularity. Urethral substitution using skin grafts and/or bladder mucosa resulted in a high complication rate of 32% and 37%, respectively, whereas use of buccal mucosa resulted in a 15% complication rate. CONCLUSIONS: Preoperative psychological counseling and discussion with other parents or patients were helpful. We no longer use free skin grafts and/or bladder mucosa for urethral substitution. Waterproofing the urethral tube was performed using either a dartos or tunica vaginalis flap. When the quality of the tissues was poor or severely scarred a 2-stage repair was performed. When resurfacing the penis local skin flaps may be insufficient or unreliable, and rotation of scrotal skin flaps or burial of the penis in the scrotum (Cecil-Culp) offers dependable skin coverage.

Adolescent↗
PubMed2005

2-stage repair in infancy for severe hypospadias with chordee: long-term results after puberty.

PURPOSE: Urinary and sexual functions were assessed in post-pubescent boys who had undergone 2-stage hypospadias repair in infancy for severe hypospadias with chordee. MATERIALS AND METHODS: A total of 44 boys who had undergone 2-stage hypospadias repair from 1985 to 1993 and who were at least 13 years old were contacted. Of the 44 boys 27 (61%) with an average age of 15.4 years (range 13 to 21) responded. Meatal locations were midshaft in 14 cases, penoscrotal in 9 and perineal in 4. Four boys had bifid scrotum and 5 had intersex disorders. Intramuscular testosterone was administered preoperatively to 15 (56%) boys. A Nesbit procedure was performed in 18 boys (67%). Average patient age at stage 2 repair was 2.3 years. Mean followup was 12.7 years (range 10.7 to 17.2). Additional surgery was performed for diverticuli in 5 cases, fistula in 3 and minor strictures in 4. Of the 27 patients 25 presented for examination and 2 responded to questionnaire only. RESULTS: All patients had normal meatal position, normal glanular anatomy, a well-defined coronal sulcus, normal cylindrical shafts without extra skin and well-defined penoscrotal junctions. Ten boys (40%) had minor spraying of stream, all stood to void and 10 (40%) milked the urethra after voiding. None had chordee. Twenty patients were able to ejaculate and 9 (42.9%) had to milk the ejaculate. Two patients (7.7%) had minor pain with erection. All subjects were satisfied with urinary, erectile and ejaculatory functions, and 23 (92%) were pleased with appearance. CONCLUSIONS: The 2-stage approach for severe hypospadias results in excellent function, cosmesis and patient satisfaction after puberty, with no chordee. Minor voiding and ejaculatory problems are to be expected. Late complications are rare. The use of extragenital skin to either primarily repair or salvage a "cripple" has not been necessary.

Adolescent↗
PubMed2004

Failed back syndrome.

BACKGROUND: Failed back syndrome (FBS) is a well-recognized complication of surgery of the lumbar spine. It can result in chronic pain and disability, often with disastrous emotional and financial consequences to the patient. Many patients have traditionally been classified as "spinal cripples" and are consigned to a life of long-term narcotic treatment with little chance of recovery. Recent progress in our understanding of this complex condition, as well as advances in surgical and medical treatment, have offered hope for improved outcomes and quality of life for these patients. REVIEW SUMMARY: This study explores the complex and multifactorial etiology of FBS, including reasons for surgical failure, indications for surgical revision, and adjunctive treatments such as pain management procedures. CONCLUSIONS: Despite extensive work in recent years, FBS remains a challenging and costly disorder. Recent advances in surgical reconstruction, rehabilitation, and pain management technique offer hope for patients with this painful and disabling condition.

Humans↗
PubMed2004

Ice storm-related carbon monoxide poisonings in North Carolina: a reminder.

Severe winter weather, such as ice storms, that results in loss of electrical power, is frequently mentioned as a contributing factor in acute carbon monoxide (CO) poisoning. However, in our literature review, such events are infrequently reported. This article reports on such an event in which more than 200 patients were evaluated and treated at a single facility because of the crippling effects of an ice storm leading to prolonged loss of power and subsequent catastrophes with alternative heating and cooking sources. One hundred seventy-six patients were treated and subsequently released after Emergency Department-based treatment for CO exposure, and three patients were admitted. Eighteen patients were treated with hyperbaric treatments and discharged. Three others left before treatment was completed. Three cases representing varying levels of severity at presentation leading to differing treatment algorithms are discussed to demonstrate a suggested clinical decision pathway in the treatment of unintentional CO poisoning.

Adult↗
PubMed1996

In situ fixation of the slipped capital femoral epiphysis with a single screw.

We made a retrospective analysis of a consecutive series of patients with slipped capital femoral epiphyses (SCFE) surgically treated at the Shriners Hospitals for Crippled Children at Lexington, Kentucky. The clinical outcome was correlated to the number of pins and the incidence of chondrolysis, ischemic necrosis, and progressive slipping after the initial fixation. An evaluation made of the Walters-Simon theoretic pin penetration formula was of no value in this series. We compiled results in the medical literature and compared them with ours; all information indicated that a single pin through an anterior portal significantly reduces complications and provides reliable fixation. However, ischemic necrosis still occurs in the unstable slip, although at a lesser rate than in cases treated with multiple pin fixation.

Bone Screws↗
PubMed1984

Genetic study of an orthopedic referral center.

A prospective study of patients seen at the Shriners Hospital for Crippled Children, Houston Unit, showed that there is a substantial genetic component to orthopedic disorders. A mean of 25% of new patients and 46% of specialty clinic patients had orthopedic diseases of genetic etiology. The respective single gene component was 7.2% and 12.9%. Rare disorders were observed, and some of these are discussed. The results indicate that genetic counseling should be an integral part of orthopedic care.

Adolescent↗
PubMedDate not supplied

Surgical management of arthrogryposis in the upper extremity.

Arthrogryposis multiplex congenita is a rare congenital disorder in which the extremities are affected with severe resistant musculoskeletal contractures. In the upper extremity, these deformities typically consist of internal rotational contractures of the shoulder, extension deformity of the elbow, flexion and ulnar deviation of the wrist, thumb-in-palm deformity, and digital interphalangeal joint flexion contractures occurring singularly or in combination. At the Houston Unit of the Shriners Hospital for Crippled Children, during the interval from 1962 to 1982, 25 patients underwent 56 operative procedures to correct upper-extremity deformities due to arthrogryposis multiplex congenita. Functional improvement was observed clinically and reported subjectively by the patients or their parents in 75% of the cases.

Arthrogryposis↗
PubMedDate not supplied

Spinal deformity in Marfan syndrome.

Fourteen patients with classic Marfan syndrome and spinal deformity treated at Texas Scottish Rite Hospital for Crippled Children (Dallas, TX) after 1975 have been conservatively treated to maturity or are at least 2 years after spinal fusion. Brace treatment was successful in one of nine patients. Nine patients underwent fusion. Three had a solid fusion, two lost 20 degrees of correction, and four required revision for multiple pseudarthroses. These latter four all had or developed a kyphotic deformity. Although Marfan syndrome patients can achieve solid fusion, they require massive bone graft, secure segmental internal fixation, and careful observation for pseudarthrosis. Kyphotic deformities require anterior fusion.

Adolescent↗
PubMed1992

Multiple sclerosis: will it come between us? Sexual concerns of clients and their partners.

Multiple sclerosis (MS), an unpredictable neurologic disease, is sometimes called the crippler of young adults. This crippling effect often involves sexual functioning. Sexuality is an issue that is frequently difficult for clients, their partners and health care providers to discuss. To address this difficulty, a teaching pamphlet entitled, MS ... Will it Come Between Us? was produced. Open communication and creativity are helpful in dealing with problems frequently encountered by clients with MS: fatigue, impotence, decreased vaginal lubrication, bladder and bowel dysfunction. The pamphlet helps clients with these issues as well as the basics of sexuality as a whole.

Adaptation, Psychological↗