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At least 541 records · Page 30Linked to original sources
PubMed2001

Lung transplantation versus lung volume reduction as surgical therapy for emphysema.

There are currently two surgical therapies aimed at crippling, end-stage emphysema: lung transplantation and lung volume reduction surgery (LVRS). Unfortunately, most emphysema patients are poor candidates for any surgical intervention. The authors favor a meticulous selection process in which indications and contraindications are considered and the best solution is devised for each patient. Patients with ideal circumstances for LVRS--hyperinflation, heterogeneous distribution of disease, FEV1 of more than 20%, and normal PCO2--are offered LVRS. Patients with diffuse disease, low FEV1, hypercapnia, and associated pulmonary hypertension are directed toward transplantation. LVRS has not been a satisfactory option for patients with a1-antitrypsin deficiency, and we prefer a transplant in most of these patients. With these considerations, we find that few patients are serious candidates for both procedures. Combinations of lung volume reduction and lung transplantation, simultaneously or sequentially, are possible but rarely necessary.

Contraindications↗
PubMed1998

Refinements in buccal mucosal grafts urethroplasty for hypospadias repair.

We evaluated our experience with buccal mucosa in urethral reconstruction for complex hypospadias repair in a total of 62 children operated on at our institution since 1990 at a mean follow-up of 23 months. After the graft had been taken from the lower lip, with possible extension to the inner cheek, complete thinning of the graft was performed. The graft was then sutured in an onlay fashion to the urethral plate, and suprapubic urinary diversion was carried out for 3 weeks. The overall complication rate in this rather complex series of patients, including 26 "hypospadias cripples," was 13%; the complications involved 4 fistulas, 3 cases of graft necrosis, and only 1 meatal stenosis during the follow-up period. These favorable results prompted us to use buccal mucosa onlays as our current method of choice for urethral reconstruction with avoidance of tubularized repairs. The definite value of this technique, however, will become evident only after a longer follow-up period and confirmation of these favorable results.

Adolescent↗
PubMed2000

Informational suppressor alleles of the eEF1A gene, fertility and cell degeneration in Podospora anserina.

Mutations that increase readthrough at a UGA stop codon (informational suppressor mutations) were created in the gene (AS4) that encodes translation elongation factor eEF1A in the filamentous fungus Podospora amserina. The results strongly suggest that the net charge of the eEF1A protein controls the accuracy of translation. Physiological analysis of the mutant strains shows that some of the alleles dominantly increase life span, while only one drastically modifies fertility. This exceptional allele (AS4-56) causes a wide array of phenotypes, including a new growth cessation phenomenon that is different from Senescence or Crippled Growth, previously known degenerative syndromes that are both controlled by AS4. The data emphasise the fact that eEF1A exerts a complex control over cellular physiology.

Alleles↗
PubMed2005

Long-term clinical, functional and radiological outcome 21 years after posterior or posterolateral fusion in childhood and adolescence isthmic spondylolisthesis.

Long-term radiological studies have shown that a high rate of fusion can be achieved with posterolateral spondylodesis. Radiological findings, however, do not always correlate with patient satisfaction and outcome. No studies have been conducted on the long-term results of functional outcome, including spinal mobility and trunk strength measurements, after operative treatment of spondylolysis and spondylolisthesis, as compared with the reference population. Of 129 consecutive patients with isthmic spondylolisthesis operated on with spondylodesis between 1977 and 1987, 107 (83%) participated in the study. Posterior spondylodesis was performed in 29 (27%) patients and posterolateral spondylodesis in 78 (73%) patients. The average follow-up time was 20.9 years (range 15.1-26.2 years). Radiographs obtained preoperatively and at the 2-year and final follow-ups were assessed for quality of the fusion and degenerative changes. Outcome was assessed at the last follow-up by physical examination, spinal mobility and non-dynamometric trunk strength measurements, and calculation of Oswestry disability index (ODI) scores. The fusion rate was 66% after posterior fusion and 83% after posterolateral fusion. Degenerative changes in the lumbar intervertebral discs above the fusion level were noted in 13 (12%) patients. At the final follow-up 14% of patients reported back pain often or very often. The mean ODI score was 7.6 (0-68). Moderate disability was found in 6% of patients and severe disability in 1%; one patient was crippled. No correlation was found between disc degeneration or solidity of the fusion and the ODI score. Non-dynamometric trunk strength measurements corresponded with the reference values. Lumbar flexion, but not extension, was diminished when compared with that of the reference population. The overall long-term clinical outcome is good in patients with spondylolysis and spondylolisthesis operated on with posterior or posterolateral fusion. The clinical and radiological outcomes do not, however, appear to correlate with each other. Lumbar flexion is diminished, but the patients perform, on average, as well as the general population in non-dynamometric trunk strength measurements.

Adolescent↗
PubMed2001

The use of allograft (and avoidance of autograft) in anterior lumbar interbody fusion: a critical analysis.

The aim of this report is to analyze the validity of allograft in anterior lumbar interbody fusion. Forty-three patients underwent anterior lumbar interbody fusion using allograft in the period between 1995 and 1998. All suffered from crippling chronic low back pain with or without sciatica. Discogenic disease was verified in 40 cases by discography. All patients were investigated preoperatively with magnetic resonance imaging (MRI). The surgical technique is described. Follow-up radiographs were performed postoperatively, then at 1.5, 3, 6 and 12 months, as required. Radiological fusion was confirmed in all single-level fusions (100%, n=24). In two-level fusions the rate was 93% (n=28/30). However, radiological union could only be confirmed in 11 of the 12 levels in the three-level fusions. Allograft offers a better alternative to autograft for anterior lumbar interbody fusion. Donor site morbidity is avoided, hospital stay is shorter and fusion rates are satisfactory.

Bone Transplantation↗
PubMed1998

Blastomycosis of the lumbar spine: case report and review of the literature, with emphasis on diagnostic laboratory tools and management.

We report on the conservative and surgical management of a patient with blastomycosis of the lumbar spine, causing severe and crippling deformity. The diagnosis was made through biopsy. Curative removal, reconstruction and realignment of the spine were achieved. Imaging modalities were highlighted, with a detailed discussion of the histology and conservative and surgical management. We emphasize the importance of early, aggressive treatment of blastomycosis to prevent deformity and disability, and to enable identification of the best management of a destructive lesion with deformity. This case demonstrates that empirical treatment should not be used in cases of unusual sinus and abscess locations. Specific diagnosis and early treatment are indicated to prevent dreadful complications and spinal deformity resulting from blastomycosis. Aggressive antifungal therapy can cure the disease but does not control complications related to deformity. The latter can only be addressed by surgical reconstruction. We review the literature of surgical treatment, focusing on abscess drainage, bone fusion and posterior instrumentation in the absence of addressing the deformity component.

Adult↗
PubMed2006

Enzyme replacement therapy and bony changes in Egyptian paediatric Gaucher disease patients.

BACKGROUND: In Gaucher disease, the infiltration of the bone marrow by glucocerebroside-laden macrophages (Gaucher cells) triggers a diverse pattern of skeletal disease that results in crippling complications. Reliable ascertainment of the severity and pattern of skeletal disease is essential to determine disease status and the response to enzyme replacement therapy (ERT). Although there is ample documentation of reversal of haematological and visceral disease by ERT, there is a paucity of data on skeletal response to ERT in children. AIM: To delineate the pattern of bone disease in children with Gaucher disease in Egypt and to evaluate its response to ERT. METHOD: Twenty-two children with Gaucher disease were treated with ERT. Phenotyping by clinical, laboratory and radiological criteria was performed at baseline and following 11.2 +/- 4 months of ERT. Genotyping for glucocerebrosidase (GBA) mutations was performed by gene sequencing, and genotype-phenotype correlations were performed.Results. Two-thirds of the patients were from consanguineous pedigrees and 14/22 patients were homozygous or compound heterozygous for L444P and D409H mutations. Bone involvement was detected by plain radiology in 11 children (50%) and in 16 (73%) by magnetic resonance imaging (MRI). There was no correlation of severity of bone involvement and GBA genotype. ERT ameliorated bone disease: 10 of the 11 children with abnormal radiographic findings at baseline showed improvement in skeletal lesions; while 9/16 showed improvement of marrow disease by MRI. Radiographic sensitivity and specificity were 62% and 82% compared to MRI for detection of bone involvement in this patient population. At baseline, bone pain was present in 5 patients and ERT resulted in complete symptomatic remission in all of them. ERT was associated with significant improvement in growth parameters and amelioration of haematological and visceral involvement. CONCLUSION: Symptomatic and radiological skeletal disease is common in children with Gaucher disease in Egypt. MRI is the most accurate technique for detecting early skeletal involvement. There was no correlation between severity of skeletal involvement and GBA genotype. ERT was effective in ameliorating radiological manifestations of skeletal disease and achieving complete remission of bone pain.

Adolescent↗
PubMed2003

Two-stage repair for proximal hypospadias: a reappraisal.

Great advances have been made in surgery for the correction of hypospadias. Proximal hypospadias remains the greatest challenge, but, despite many innovations and much progress, surgery can fail. Many authorities have introduced single-stage techniques, which have the purported advantage of correcting the defect with minimal hospitalization and family inconvenience. However, the wider published experience with these approaches would suggest that the ideal single-stage procedure has yet to be devised. A substantial number of children undergoing a single-stage procedure will have to undergo further surgery. A small, but irreducible number of patients will be rendered hypospadias "cripples." A number of these children will be dissatisfied as adults because of cosmetic or functional deficiencies. A two-stage approach for correction of proximal hypospadias and severe chordee remains the most credible and reliable solution for many of these patients. Pediatric urologists should maintain familiarity with these techniques and continue to advocate them for a select group of patients.

Child↗
PubMed2005

Prostatitis: epidemiology of inflammation.

Approximately 8 million prostatitis-related outpatient visits per year have been reported worldwide. Only a small percentage presents with bacterial prostatitis. Chronic pelvic pain syndrome/prostatodynia is a frustrating disease for patients and caregivers. This paper is an attempt to analyze causes and types of inflammation and to determine if inflammation may have an impact on treatment and outcome. Is inflammation even a relevant factor in the diagnosis of chronic pelvic pain syndrome? Do we need to begin to look for different factors involved in the development of this often crippling disease?

Autoimmune Diseases↗
PubMed1975

Cor pulmonale (pulmonary heart disease): present-day status.

In reviewing the present-day status of cor pulmonale, it is clear that considerable progress has been made in almost all instances of the disease. It is clearly a preventable form of heart disease in most cases and it is treatable and curable in its most common form, i. e., in COPD. One must agree with Petty, that today we have effective means of caring for the majority of respiratory cripples. Therapy for lung disease now appears even to have reduced the expected rate of pulmonary function deterioration in some patients. Surely with improved gas exchange and early detection of respiratory insufficiency the outlook for patients with respiratory diseases leading to cor pulmonale is better today than it was 30 years ago.

Adult↗
PubMed1977

Management of carcinoma in situ of the vulva.

Concern about multicentricity and occult invasion has led authorities to recommend total vulvectomy in the management of carcinoma in situ of the vulva (VCIS). Of these considerations, only occult invasion has sufficient import to contraindicate a more conservative therapeutic approach. VCIS is being diagnosed with increasing frequency in young women for whom the deforming and sexually crippling effects of vulvectomy are especially repugnant. Because of its distinctive success in localizing preinvasive and early invasive squamous neoplasia of the cervix, colposcopy and directed biopsy were employed in evaluating all patients seen in our vulva clinic since 1971. Of 27 consecutive patients considered to have VCIS, 24 were treated either by local excision, skinning vulvectomy, topical 5-fluorouracil (5-FU), or cryosurgery. In no instance was occult invasion missed on pretreatment evaluation, and only one patient has developed a new in-situ lesion following conservative surgical therapy. Topical 5-FU therapy was unsuccessful in six of six cases. These results demonstrate that total vulvectomy for VCIS can be replaced successfully with more conservative operations.

Adult↗
PubMed1989

'Calcium-activated' intracellular calcium elevation: a novel mechanism of osteoclast regulation.

The osteoclast is unique in its capacity to resorb bone. An unbalanced increase in this activity causes osteoporosis, a crippling bone disease that poses a major public health problem. Despite this, our understanding of osteoclast regulation is very limited. Calcitonin is the only known physiological inhibitor of osteoclast function. We demonstrate here for the first time that the concentration of calcium ions at the resorptive site directly regulates osteoclast function by modulating the intracellular free calcium concentration. This represents an important feedback mechanism of osteoclast control.

Acid Phosphatase↗
PubMed1983

Decreased horseradish peroxidase labeling in deafferented spinal motoneurons of the rat.

It has been suggested that the incorporation and retrograde transport of horseradish peroxidase (HRP) were linked to the level of neuronal activity. Therefore one could postulate that the motor impairment resulting from dorsal rhizotomy affects the HRP labeling of spinal motoneurons in the absence of morphological damage to the motor system. This hypothesis was tested in the adult rat by sectioning bilaterally the L3-L5 dorsal roots. 2-18 months after surgery, the L4 radicular nerve was immersed in a solution of HRP. Labeled motoneurons were counted together with the motor axons of the L4 ventral root and results were compared with values obtained in paired controls. Deafferentation resulted in a crippling deficit of lower limb movements with disuse atrophy of muscle fibers but had no effect on the fiber population of the sciatic nerve and the L4 ventral root. Whereas in normal animals the L4 HRP-labeled motoneurons represented 71.9-98.3% (average 85.4) of the motor axonal counts, in animals studied 4, 12 and 18 months after dorsal rhizotomy, the number of motoneurons containing HRP granules constituted only 20.1-55.7% (average 46.2) of the number of motor axons and many of the labeled cells were faintly stained. These findings, which may reflect either a decreased retrograde transport of HRP in deafferented motoneurons or an increased turnover of the enzyme in the cell body, call attention to the possibility that the degree of activity in neuronal pathways influences HRP labeling.

Afferent Pathways↗
PubMed1975

Studies on the carrier state in X-linked recessive (Duchenne) muscular dystrophy.

Observations in 12 normal women and 12 female carriers of X-linked recessive Duchenne muscular dystrophy (DMD), of whom 4 had symptoms and 8 had none, were compared between all 4 groups and with those in 2 DMD boys, one active and one crippled. Carrier symptoms were readily ascertained by systematic examination. Measurement of both lower legs in all 24 women showed neither calf enlargement nor asymmetry in carriers beyond normal variation. Two DMD carrier daughters were noted of the same DMD carrier mother but by different fathers. Whole body counting showed the biological half-life of previously administered 86Rb to be much reduced in DMD, but no differences were found between normal women and any group of carriers. The test is thus valueless for carrier detection, and reasons are given why it should be so. Simultaneous measurement of total body K+, with subsequent determination by isotope dilution of total body water as 3H2O space and extracellular water as NH4 82Br space, showed increased intracellular water and reduced intracellular K+ concentration in all carriers, as if due to osmotic causes, with actual loss of muscle mass and slight diminution of serum K+ in the 4 symptomatic carriers only. Because of diurnal and other variations, the means and standard deviations for six serum enzymes from six fortnightly assays in all subjects were used to measure precise individual status. Their coefficients of variation were abnormal only in symptomatic carriers and ambulant DMD, easily overtexed by even accustomed exertion. This is shown to support previous propositions on the pathogenesis of DMD and the escape of muscle cell content into the circulation.

Adolescent↗
PubMed1992

On how hydrolysis at the 3' end is prevented in the splicing of a sequentially folded group I intron.

We propose a dynamic model for the competition between exon-exon ligation and 3'-end hydrolysis valid for sequentially folded pre-mRNA introns of group I. This model accounts for the delay in the formation of conserved helix P10 until the 5' exon has been cleaved, a requirement to prevent hydrolysis at the 3' end of the intron. The model is rooted on computer simulations whereby the pre-mRNA searches for its structure as it is being transcribed. Thus, a competing interaction, engaging the internal guiding sequence, occurs initially and prevents P10 from forming until the 3' end of the 5' exon is habilitated as a nucleophilic agent. It is further shown that a destabilization of the competing interaction invariably leads to 3' hydrolysis, crippling the splicing capability of the intron. The results may be probed by site-directed mutagenesis.

Base Sequence↗
PubMed1989

Steroid-related osteonecrosis in inflammatory bowel disease.

Osteonecrosis is a serious complication of steroid therapy characterized by death of all the cellular elements of bone. We describe a series of patients with steroid-induced osteonecrosis in inflammatory bowel disease. Seven of 161 patients (4.3%) treated with corticosteroids for inflammatory bowel disease over a 10-yr period developed osteonecrosis. The median age at the onset of inflammatory bowel disease was 20 yr and for osteonecrosis the median age was 28 yr. Patients had received steroids for a mean duration of 42 wk with a mean daily dose of 26 mg/day and a mean cumulative lifetime prednisone dose of 7 g. Osteonecrosis occurred within 6 mo of the last administration of steroid in all patients. It presented with joint pain in the hip or knee and was frequently mistaken for the arthralgia of steroid withdrawal or the arthropathy of inflammatory bowel disease. Multiple joints were involved in 6 of the 7 patients. Surgery for the joint disease was required in 4 of the 7 patients. The median duration of follow-up was 2 yr. Five of the 7 patients continued to have significant joint pain and disability that limited their activity. We conclude that inflammatory bowel disease predisposes to steroid-induced osteonecrosis. The age of patients is younger, and the dose and duration are considerably lower than that reported for steroid-induced osteonecrosis in other disease states. Bone scans or magnetic resonance imaging should be performed in patients with joint pain who are receiving or have recently received corticosteroid therapy. Early detection and treatment may prevent the crippling long-term complications of this disease.

Adult↗
PubMed1976

Erythrocyte spectrin peak II phosphorylation in Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a rapidly progressive crippling disease of young boys that is inherited as an X-linked recessive trait. Previous studies have demonstrated the usefulness of erythrocyte studies in exploring membrane abnormalities in inheritied muscular dystrophy. Erythrocyte spectrin peak II protein (m.w. equivalent to 220,000) was more highly phosphorylated under initial rate conditions in DMD than in controls. The extent of peak II phosphorylation was greater in DMD erythrocytes and a Na+ stimulated peak II phosphorylation effect (Avruch and Fairbanks 1974) was not found to account for the differences between DMD and controls. The phosphorylated state of spectrin proteins in the membrane was evaluated and no differences in DMD could be measured. The maximal transfer of phosphate from differences in DMD could be measured. The maximal transfer of phosphate from [gamma-32P]ATP to spectrin peak II accounts for approximately 5-10% of the total phosphate content of spectrin.

Adenosine Triphosphate↗