Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “RUBY”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 991 records · Page 55Linked to original sources

Compartment syndrome after elective revascularization for chronic ischemia. A case report and review of the literature.

Compartment syndrome is a rare but serious complication of vascular reconstruction. It most commonly occurs following revascularization of an acutely ischemic extremity. This case of compartmental syndrome following bypass surgery for chronic arterial insufficiency is therefore exceedingly unusual. A review of the literature reveals only a few cases with similar presentation. The diagnosis can usually be made on the basis of clinical manifestations combined with compartmental pressure measurement. A timely, four-compartment fasciotomy can avert serious complications with minimal morbidity. Late recognition and treatment of this complication, as well as inadequate decompression, can lead to loss of limb.

Chronic Disease↗

Unilateral renal artery stenosis seen initially as severe and symptomatic hypokalemia. Pathophysiologic assessment and effects of surgical revascularization.

Hypokalemia is an uncommon presentation of renovascular hypertension. Although renal artery stenosis has been associated with hypokalemia secondary to hyperreninemic hyperaldosteronism, few reports have actually evaluated the pathophysiologic changes in such a patient with renovascular hypertension. We studied a patient before and after surgical revascularization who presented with severe hypertension and marked, symptomatic hypokalemia. Before surgery, the patient had excessive urinary potassium secretion, markedly increased secretion of renin after captopril stimulation, and mild secondary hyperaldosteronism. Postoperatively, the patient's blood pressure decreased moderately and the serum and urinary potassium values normalized. After revascularization, plasma renin activity both before and after captopril stimulation and serum aldosterone levels decreased markedly. These findings demonstrate that renovascular hypertension may rarely present with symptomatic hypokalemia secondary to excessive aldosterone secretion. Improvement in the renal ischemic state is accompanied by rapid correction of the metabolic disturbances associated with hyperreninemic hyperaldosteronism.

Aldosterone↗

Metastatic neuroblastoma in children older than one year: prognostic significance of the initial metaiodobenzylguanidine scan and proposal for a scoring system.

BACKGROUND: Metaiodobenzylguanidine (mIBG) is a guanethidine analog that has demonstrated a high sensitivity and specificity in detecting bone metastases in about 90% of metastatic neuroblastomas. However, the predictive value of initial mIBG scan in neuroblastoma patients older than 1 year of age regarding response to initial chemotherapy has yet to be ascertained. Therefore, a scoring system for grading the positivity of mIBG scans was devised and applied in a retrospective study in an attempt to determine whether this score had a prognostic value in neuroblastoma patients older than 1 year of age at diagnosis. METHODS: Eighty-six children, older than 1 year of age, with metastatic neuroblastomas were homogeneously treated and had a mIBG scan performed at diagnosis and following the induction regimen to assess bone metastases. Each mIBG scan was assigned a reproducible score and the predictive value of the initial mIBG score was assessed in order to evaluate response to induction regimen. RESULTS: The relative risk of failing to achieve complete remission after four courses of induction therapy was 6.9 times higher in patients who had more than four mIBG spots at diagnosis. A multivariate analysis including the established prognostic factors revealed that the initial mIBG score was the only significant factor (P < 0.001). CONCLUSIONS: The initial mIBG scan is of prognostic significance to predict response to chemotherapy for metastatic neuroblastoma in children older than 1 year of age. A prospective study comparing this initial mIBG score with other recently established prognostic factors is warranted.

3-Iodobenzylguanidine↗

The treatment of neuroblastoma with intraspinal extension with chemotherapy followed by surgical removal of residual disease. A prospective study of 42 patients--results of the NBL 90 Study of the French Society of Pediatric Oncology.

BACKGROUND: Neuroblastoma is the most common malignant cause of spinal compression in the pediatric population. More than 30% of patients who are impaired prior to treatment remain impaired after the completion of therapy. Those who do not improve after decompressive laminectomy may go on to develop severe delayed spinal deformities. METHODS: To decrease the long term sequelae of routine neurosurgical intervention for all intraspinal extensions of neuroblastoma, the French NBL 90 Study was formulated to use chemotherapy as a first-line treatment for all nonmetastatic neuroblastomas with intraspinal extension. Neurosurgical decompression and excision was recommended only for patients demonstrating rapid neurologic deterioration. RESULTS: The overall survival of the 42 patients registered was 97%. Initial neurologic impairment was present in 27 patients (64%), including 11 with paraplegia. Thirty-two patients received chemotherapy as first-line treatment. Complete regression of the intraspinal component was observed in 13 patients and partial regression of greater than 50% of the initial volume in 5 patients. Of 19 evaluable patients presenting with a neurologic deficit and treated with primary chemotherapy, recovery was completed in 11 and partial in 3. Four patients failed to recover from long-standing pretreatment paraplegia. Only one patient worsened during therapy, and recovered completely after emergent neurosurgical intervention. Seven patients underwent initial neurosurgical procedures; six had a neurologic deficit and five recovered completely, including all three who presented with acute onset of paraplegia. Three patients had extraspinal surgery as exclusive treatment. Six patients (15%) suffered severe neurologic sequelae. Only one of the patients who underwent surgery required spinal stabilization for progressive deformity, but follow-up is limited. CONCLUSIONS: By treating patients with dumbbell neuroblastoma initially with chemotherapy, the authors were able to reduce the size of the intraspinal mass in 58% of patients, improve partial neurologic deficits in 92%, and avoid neurosurgical decompression in 60%. Neurologic deficits also improved in 83% of patients requiring emergent neurosurgical intervention.

Adolescent↗

Respiratory function in children undergoing bone marrow transplantation.

We conducted a prospective study of respiratory function in children undergoing bone marrow transplantation (BMT) for onco-hematological disorders. Each child was evaluated before and 100 days after BMT. The investigations included clinical examination, chest X-ray, and pulmonary function tests (PFT) to determine: slow vital capacity (VC), functional residual capacity (FRC), total lung capacity (TLC), forced expiratory volume in 1 s (FEV1), carbon monoxide diffusing capacity (DLCO), ratio of residual volume (RV) to TLC, and FEV1/VC. The values obtained before and after BMT were compared to predicted values, and the post-BMT values were compared to the pre-BMT values (Student's t-test). From 1986 to 1995, 77 children underwent BMT, of whom 39 were available for testing. The pre-BMT VC (P = 0.0234) and DLCO (P < 0.0001) were lower and FRC higher (P < 0.0001) than predicted values. After BMT, the VC (P = 0.004), TLC (P = 0.044), and FEV1 (P = 0.012) were lower, and the RV/TLC ratio was higher (P = 0.043), compared with pre-BMT data. The observed respiratory abnormalities were not clinically relevant. The only identifiable risk factor for a decrease in lung function was age at BMT. This study shows that some lung dysfunction may be present before BMT and be further altered by BMT. This stresses the need for longitudinal respiratory monitoring and follow up to detect such dysfunctions and to insure an optimal treatment program for these children.

Age Factors↗

Patient self-assessment of complications and quality of life after conformal neutron and photon irradiation for localized prostate cancer.

Although neutron irradiation for prostate cancer has been associated with significant morbidity, pilot data in patients with early stage disease suggested that conformal neutron and photon irradiation was well tolerated without severe complications. A self-assessment questionnaire was mailed to the first 83 patients treated with conformal neutron and photon irradiation to objectively evaluate the impact on quality of life of this regimen. In total, 75 patients (90%) returned the completed questionnaire. These patients had received either 9 neutron Gy (N Gy) plus 38 photon Gy (50 patients) or 10 N Gy plus 38 photon Gy (33 patients) for stage T1/T2 N0 M0 prostate cancer (Gleason score < or = 7). The irradiated volume included the prostate and seminal vesicles with a 1.5 cm margin. Neutrons were delivered as a boost to the prostate only with a non-axial four field beam arrangement. Approximately 50% of the dose to the prostate was from neutron irradiation. Follow-up ranged from 6 to 25 months (median 13 months). The questionnaire used was a validated quality of life instrument used previously in patients treated with surgery or radiation. Prior to irradiation, 29% of patients reported urinary symptoms and 11% had prostate surgery (TURP). At follow-up, 23% reported persistent urinary symptoms. Of these, 21% dripped a few drops of urine, 3% used pads but none leaked > 1 tablespoon/day. One patient underwent surgery to dilate a urethral stricture. Although 55% of patients had gastrointestinal symptoms during radiation therapy, only 26% had persistent symptoms at the time of questioning. These included minor rectal bleeding in 20% and significant hematochezia in 3%. Of the 85% of patients able to obtain full or partial erections prior to irradiation, 87% maintained their potency. Fifteen percent sought treatment for impotence. Overall, at last follow-up, 84% felt little or no physical discomfort, 91% were very satisfied with their treatment, and 97% would choose radiation therapy again. This patient self-assessment questionnaire confirmed that this regimen of conformal neutron and photon irradiation resulted in levels of chronic toxicity acceptable to the patient.

Abdominal Pain↗

Localised and unresectable neuroblastoma in infants: excellent outcome with primary chemotherapy. Neuroblastoma Study Group, Société Française d'Oncologie Pédiatrique.

PROCEDURE: Infants with neuroblastoma (NB) were assessed according to INSS recommendations, including MIBG scan and extensive bone marrow staging to eliminate metastatic spread. Patients with unresectable tumour received chemotherapy, including two courses of carboplatin-etoposide (CE) and two of vincristinecyclophosphamide-doxorubicin (CAdO). Post-operative treatment was to be given only in infants with MYCN amplification. Between 1990 and 1994, 52 consecutive children were registered. RESULTS: Among the 44 patients who received CE as a first course, the response rate was (66%) and the primary could be removed in all children but one, who was in remission. The toxicity was mainly haematological and was always manageable. The 5 year overall survival (OS) and event-free survival (EFS) were 94 and 90 +/- 8%, respectively, with a median follow-up of 48 months. The outcome of infants with no MYCN amplification was excellent; OS and EFS were, respectively, 97 and 94%. CONCLUSIONS: Chemotherapy allows surgical excision and excellent outcome in infants with localised and unresectable NB. Less intensive Chemotherapy should be investigated in such patients.

Antineoplastic Combined Chemotherapy Protocols↗

Kinetic, hormonal and clinical studies with aminoglutethimide in breast cancer.

Approximately one-third of patients with metastatic breast carcinoma respond to surgical ablative therapy but the morbidity associated with these procedures has limited their use to highly selected patients. Consequently, a chemical method of adrenal suppression was developed using a potent inhibitor of adrenal steroid synthesis, aminoglutethimide, in combination with a synthetic glucocorticoid, dexamethasone. While this regimen effectively blocked adrenal function, it was complicated by a drug interaction in which aminoglutethimide accelerated the metabolism and reduced the bioavailability of dexamethasone. To overcome this problem, a new regime using aminoglutethimide and hydrocortisone, a glucocorticoid less susceptible to altered metabolism, was developed. Kinetic studies confirmed that aminoglutethimide does not interact with hydrocortisone to alter its rate of metabolism. Hormone measurements established that 1000 mg of aminoglutethimide and 40 mg of hydrocortisone daily suppressed DHA-sulfate, androstenedione, estrone, estradiol and aldosterone to a greater extent than the prior protocol using aminoglutethimide and 2-3 mg of dexamethasone. Patients experienced objective tumor regression with equal frequency while receiving the aminoglutethimide-hydrocortisone regimen or aminoglutethimide and dexamethasone and the overall rate of response in 50 evaluable patients was 38%. Side effects occurred frequently in the first few weeks of treatment but disappeared nearly uniformly thereafter. The present aminoglutethimide-hydrocortisone regimen is simple, non-toxic, effective in inhibiting estradiol synthesis and capable of inducing tumor regression as frequently as previously reported with adrenalectomy.

Adrenocorticotropic Hormone↗

Morphologic and biochemical characteristics of isolated and cultured pancreatic ducts.

Rat and hamster pancreatic ducts were isolated by digestion with collagenase plus chymotrypsin and were cultured for eight weeks in an agarose matrix. Freshly isolated and cultured ducts were characterized morphologically and biochemically. The in vivo morphology of the ducts was maintained in vitro, although certain differences were noted. Both interlobular and intralobular ducts could be identified. gamma-Glutamyltranspeptidase and Mg-ATPase were stable enzymatic activities of the ducts of both species; alkaline phosphatase persisted only in the hamster ducts. Carbonic anhydrase and (Na + K)ATPase were minor activities of the rat ducts. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis of the rat ducts suggested that actin was the major duct peptide and that the major zymogens were greatly diminished. These results demonstrate that pancreatic ducts can be maintained in vitro and can be used for biochemical studies of this minor pancreatic tissue type.

Adenosine Triphosphatases↗

Recurrent renal salt wasting in a child treated with carboplatin and etoposide.

BACKGROUND: Nephrotoxicity of carboplatin is rare, especially in children with normal renal function. A 3-year-old boy had localized esthesioneuroblastoma and received 2 courses of carboplatin (200 mg/m2/day during a 1-hour infusion for 3 consecutive days) associated with etoposide (150 mg/m2/day after carboplatin). Because of a good tumor response, a second course was given 21 days later. Complete surgical excision and local irradiation were performed. However, the tumor recurred a few months later, and the patient subsequently died of the disease. METHODS: Renal function initially was assessed by standard baseline chemistry and technetium-panetetic acid (Tc-DTPA) clearance. Follow-up included ionic controls and tubular exploration during the episodes of hyponatremia. RESULTS: Hyponatremia occurred 4 days after completion of the first course and resolved after intravenous supplementation. It recurred 20 days after the second course despite salt and magnesium prehydration and posthydration and resolved 17 days later. No glomerular dysfunction was noticed. The association of urinary bicarbonate loss with elevated N-acetyl-beta-glucosaminidase suggested a proximal tubular damage. CONCLUSIONS: Standard doses of carboplatin may lead to recurrent renal salt wasting in children with initially normal renal function.

Antineoplastic Combined Chemotherapy Protocols↗

Reversal of low-molecular-weight heparin-induced bleeding in patients with pre-existing hypercoagulable states with human recombinant activated factor VII concentrate.

Low-molecular-weight heparins are widely employed in prophylactic and therapeutic antithrombotic regimens for venous thromboembolic events. Excessive anticoagulation with low-molecular-weight heparins rarely can precipitate catastrophic bleeding complications. Currently, there is no specific or reliable antidote that can reverse the anticoagulant effects of low-molecular-weight heparins efficiently and safely. This report describes three individuals with underlying hypercoagulable states, who developed clinically significant bleeding complications while receiving therapeutic anticoagulation with enoxaparin. All of the hemorrhagic events subsequently were safely and effectively reversed with a single intravenous bolus infusion of recombinant activated factor VIIa (RFVIIa) concentrate. Hemoglobins, prothrombin times, and clinical overt bleeding were monitored before and after the administration of RFVIIa. In all three cases, bleeding was controlled without an increase in thrombotic events. Our findings demonstrate that RFVIIa can rapidly and safely reverse the hemorrhagic adverse effects associated with excessive levels of low-molecular-weight heparin in patients with pre-existing hypercoagulable conditions and/or acute venous thromboembolism.

Adult↗

Association analysis of polymorphisms at the interleukin-1 locus in essential hypertension.

Infection with microorganisms such as Helicobacter pylori and Chlamydia pneumoniae has been associated with coronary heart disease (CAD) and hypertension (HT). Infection increases the release of pro-inflammatory cytokines, thus facilitating interactions that lead to vascular damage and other effects. We hypothesized that genetically determined differences in activity or responsiveness of cytokine(s) might contribute to HT. The interleukin-1 gene (IL1) cluster on chromosome 2q14 contains three related genes (IL1A, IL1B, and IL1RN) located within a 430-kb region. These encode IL-1alpha and IL-1beta, as well as their endogenous receptor antagonist, IL-1ra. The IL1RN gene has a penta-allelic 86-bp tandem repeat in intron 2. Allele IL1RN* 2 is associated with a wide range of chronic inflammatory and autoimmune conditions, and its combination with the -31T variant of an IL1B C(-31)T polymorphism constitutes a pro-inflammatory haplotype that leads to vigorous IL-1beta production. We therefore tested each of these polymorphisms for association with HT. Subjects were white Anglo-Celtic residents of Sydney, Australia. Frequencies of IL1B C(-31)T genotypes CC, CT, and TT were 0.50, 0.40, and 0.10 in normotensive (NT) and 0.46, 0.46, and 0.08 in HT, respectively (chi(2) = 1.2, P = 0.55). T allele frequency in NT (0.30) was similar to that in HT (0.31). For the IL1RN variant, frequencies of alleles IL1RN* 1 and * 2 and combined minor alleles * 3, * 4, and * 5 were 0.61, 0.36, and 0.03 in NT and 0.54, 0.36, and 0.10 in HT, respectively (chi(2) = 11, P = 0.004). In conclusion, no association of the IL1B C(- 31)T with HT was found, whereas combined frequency of the minor alleles of the IL1RN polymorphism was increased in the HT cohort studied.

Australia↗

Comparison of polymorphisms in the alpha7 nicotinic receptor gene and its partial duplication in schizophrenic and control subjects.

The hypothesis that the 15q13-15 region of chromosome 15 contains a gene that contributes to the etiology of schizophrenia is supported by multiple genetic linkage studies. The alpha7 neuronal nicotinic acetylcholine receptor (CHRNA7) gene was selected as the best candidate gene in this region for molecular investigation, based on these linkage findings and biological evidence in both human and rodent models. CHRNA7 receptors are decreased in expression in postmortem brain of schizophrenic subjects. A dinucleotide marker, D15S1360, in intron two of the CHRNA7 gene is genetically linked to an auditory gating deficit found in schizophrenics and half of the first-degree relatives of patients. Single strand conformation polymorphism (SSCP) and sequence analyses of DNA from schizophrenic and control individuals identified 33 variants in the coding region and intron/exon borders of the CHRNA7 gene and its partial duplication, dupCHRNA7; common polymorphisms were mapped. Twenty-one variants were found in the exons, but non-synonymous changes were rare. Although the expression of CHRNA7 is decreased in schizophrenia, the general structure of the remaining receptors is likely to be normal.

Base Sequence↗

Lack of JC viral genomic sequences in multiple sclerosis brain tissue by polymerase chain reaction.

With DNA extracted from brain specimens from 19 multiple sclerosis, 5 progressive multifocal leukoencephalopathy, 1 Alzheimer's disease, and 8 nonneurological control subjects, polymerase chain reaction was performed using nested sets of primer pairs amplifying segments of the large T and VP1 antigen-encoding sequences of JC virus. Both sequences were detected in each of the 5 brain specimens of progressive multifocal leukoencephalopathy but in none of the 19 multiple sclerosis, 1 Alzheimer's disease, or the 8 control brain specimens.

Base Sequence↗