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Biomedical subjects

R Vidal

Publications and source records attributed to R Vidal.

At least 127 records · Page 7Linked to original sources

Five-day course of granulocyte colony-stimulating factor in patients with prolonged neutropenia after adjuvant chemotherapy for breast cancer is a safe and cost-effective schedule to maintain dose-intensity.

PURPOSE: To analyze the safety and efficacy of a short course of granulocyte colony-stimulating factor (G-CSF) to maintain dose-intensity of subsequent cycles of chemotherapy after a prior episode of prolonged neutropenia, without febrile complications, in patients receiving adjuvant treatment for breast cancer. PATIENTS AND METHODS: Thirty-two patients undergoing adjuvant cyclophosphamide, methotrexate, and fluorouracil (CMF) or doxorubicin-CMF for stages I to II breast cancer were included after having chemotherapy delays due to neutropenia (absolute neutrophil count [ANC] < 1.5 x 10(9)/L) on day 22. G-CSF was administered subcutaneously on days 15 to 19 of each subsequent cycle. RESULTS: None of the patients included in this study had to be admitted to the hospital for fever and neutropenia. The median percentage of the projected dose-intensity for CMF or doxorubicin-CMF on an intent-to-treat basis was 0.994, which was significantly higher than the delivered dose-intensity before the start of G-CSF treatment (P < .0001). Patients who received concomitant G-CSF and radiotherapy achieved a similar dose-intensity as patients who did not undergo radiotherapy. Seven patients discontinued G-CSF treatment due to musculoskeletal pain. These patients had more subsequent cycle delays because of day 22 neutropenia than the 25 patients who followed the G-CSF schedule (P = .0028). CONCLUSION: A 5-day course of G-CSF in patients with prior chemotherapy delays due to prolonged neutropenia seems to be a safe and cost-effective schedule to maintain CMF or doxorubicin-CMF dose-intensity in the adjuvant treatment of breast cancer.

Adult↗

Familial meningocerebrovascular amyloidosis, Hungarian type, with mutant transthyretin (TTR Asp18Gly)

Amyloid deposits in leptomeningeal vessels, subarachnoid, subpial, and subependymal cerebrospinal regions, spinal ganglia, peripheral nerves, and some internal organs (predominantly heart and kidney) characterize a dominantly inherited disease in a Hungarian family. We found four definitely and three probably affected members in this family of 56 persons in four generations. Clinical features in all definitely diseased patients include disturbance of memory, psychomotor deceleration, ataxia, and hearing loss. In most patients there was temporary disorientation, migraine-like headache with vomiting, and tremor. Some patients had nystagmus, pyramidal signs with spastic paraparesis, hallucinations, urinary retention, and obstipation. Single patients had facial tics and sleep disorders. Progressive visual disturbance and clinically manifest polyneuropathy were absent. CSF protein was markedly elevated in all patients. CT showed characteristic symmetric calcification along the sylvian fissure; MRI after contrast administration showed prominent enhancement at the surface of the sylvian fissures, brainstem, and cerebellum. Autopsy data was available in three definitely affected patients and in one unaffected family member. Immunohistochemistry identified the amyloid deposits as of the AF (transthyretin, TTR) type; DNA studies revealed a novel TTR missense mutation at codon 18 (TTR Asp18Gly). According to clinical features, pathologic alterations, and molecular studies, this disease is a novel type of systemic familial amyloidosis with disease manifestation clinically restricted to the CNS. It is similar to the oculoleptomeningeal amyloidoses but can be clinically diagnosed by characteristic CTs and the absence of progressive visual impairment.

Adult↗

Incidence and significance of acid-fast bacilli in sputum smears at the end of antituberculous treatment.

OBJECTIVE: A group of patients with pulmonary tuberculosis (PT) who received and correctly completed antituberculous therapy were studied to determine the incidence and significance of positive acid-fast bacilli (AFB) in sputum smears at the end of this treatment. DESIGN: Retrospective chart review of persons with bacteriologically proved PT. PATIENTS: Of 1,052 patients diagnosed as having PT between 1988 and 1992, 453 who fulfilled the following criteria were included in the study: (1) diagnosis established by positive AFB and positive culture in sputum smears; (2) no previous antituberculous treatment had been received; (3) HIV serologic test results were negative; (4) treatment was correctly completed; (5) they were followed up throughout the period of treatment; and (6) expectoration was still present at the end of treatment and at least two spontaneous sputum samples could be obtained. RESULTS: Positive AFB of sputum smears were found at the end of treatment in 10 (2.2%) of the 453 patients studied. Five patients had only one positive smear, and the other five had more than one. Of these ten cases, sputum culture was negative in eight, which were considered to be unviable bacilli, and positive for nontuberculous mycobacteria in two. Clinical symptoms or worsening on chest radiograph were observed only in one patient with unviable bacilli, but they were caused by a concomitant nonspecific respiratory tract infection. CONCLUSIONS: Positive AFB smear results at the end of completed treatment regimens analyzed in this study have occurred because of unviable bacilli and nontuberculous mycobacteria colonization. The presence of more than one positive smear seems not to increase the probability of treatment failure and is more frequently due to nontuberculous mycobacteria. Results of culture can thus be awaited without the need to prolong or modify antituberculous therapy.

Humans↗

Meningocerebrovascular amyloidosis associated with a novel transthyretin mis-sense mutation at codon 18 (TTRD 18G)

We describe a novel transthyretin mutation at codon 18 where Asp is replaced by Gly (D18G) in a Hungarian kindred. This mutation is associated with meningocerebrovascular amyloidosis, producing dementia, ataxia, and spasticity. Fifty different transthyretin mutations are related to amyloid deposition, typically producing a peripheral neuropathy or cardiac dysfunction. These symptoms are absent in this family. Up to now, amyloid-beta (A beta), cystatin C, and prion proteins have been known to be deposited as amyloid in the brain, leading to stroke or dementia. With this report we establish that transthyretin amyloid deposition can also produce central nervous system dysfunction as the major clinical symptom.

Amino Acid Sequence↗

Light chain cardiomyopathy. Structural analysis of the light chain tissue deposits.

Cardiomyopathy due to monoclonal light chain deposits is a complication of plasma cell disorders. The deposits may be either fibrillar as in light chain amyloid or nonfibrillar as in light chain deposition disease. The reasons for these structural differences are still unknown. We characterized the myocardial deposits by immunohistochemical examination of sections and extraction and biochemical analysis of the tissue deposits in a patient (MCM) who died of myeloma and systemic light chain deposition disease. Amino acid sequence analysis of the extracted nonfibrillar MCM kappa-light chain reveals that it belongs to the L12a germline subset of the kappa(I) protein and contains five distinctive amino acid substitutions (three in the framework region III and two in the complementarity-determining region III) that have not been reported previously in the same positions in other kappa(I) light chains. The theoretically determined isoelectric point (pI 8.21) of the MCM light chain is high compared with the low isoelectric point of other Bence Jones proteins from subjects without light chain deposition disease. The diffuse binding to basement membranes and the high isoelectric point of the MCM kappa-light chain suggest electrostatic interaction as a possible mechanism of tissue deposition. The spatial locations of the five distinctive residues and a sixth rare substitution of the MCM protein modeled on the backbone structure of REI, a kappa(I)-soluble Bence Jones light chain of known three-dimensional structure, may be responsible for protein destabilization, partial unfolding, and aggregation leading to tissue deposition.

Adult↗

[Parainfectious transverse myelitis in an adolescent. Difficulties in the etiological diagnosis].

A diagnostic process in a 13 year-old boy with a parainfectious transverse myelitis is described. Its onset was acute, with L1-location level of lesion. After a mild improvement, a relapse happened 26 days later, with a D6 level and without subsequent recovery. It has not been found criteria for diagnosis of multiple sclerosis, nor data suggesting a vascular or ischaemic anomaly. Likewise, other causes and specific infections were rejected, being cataloguet as parainfectious etiology, due a previous viral infection before its onset.

Acute Disease↗

[Pulmonary and laryngeal tuberculosis. Study of 26 patients].

A description is reported of tuberculosis cases (TBC) with symptomatic laryngeal involvement diagnosed and treated from 1982 to 1994. Twenty-six out of 2,800 (0.9%) patients diagnosed with TBC had laryngeal symptoms. Twelve patients underwent laryngeal biopsy and the disease was diagnosed in 11; the remaining 15 patients were diagnosed on the basis of the typical lesions at laryngoscopy and resolution with specific therapy; in all of them there was a pulmonary TBC associated. The mean time of clinical laryngeal symptoms was five months and the most common symptom was dysphonia. There were factors which increased the risk for TBC in 16 patients (61.5%), alcoholism being the most common symptom in 10 (38%) patients. X-Ray examination revealed bilateral infiltrates or cavitation in 81% of patients. Sputum examination for acid-fast bacilli was positive in 15 (58%) and culture for Mycobacterium tuberculosis was positive in all of them. All patients adhered correctly to the therapeutic regimen and the clinical course was towards healing: no patient had laryngeal carcinoma after one year of follow-up. The incidence of symptomatic laryngeal TBC in our environment is low and usually presents in patients with risk factors, particularly alcoholism, with a long evolution of the disease and associated with extensive pulmonary TBC. The coexistence with laryngeal carcinoma is exceptional; therefore, when the association of laryngeal symptoms and active pulmonary TBC is present it is reasonable to reserve the laryngeal biopsy for those patients with lymph node enlargement, risk factors, or when symptoms persist after a correct therapeutic regime has been instituted.

Humans↗

Biofilm formation by Acinetobacter baumannii.

Acinetobacter baumannii, an important nosocomial pathogen is usually found on various surfaces in the hospital environment. In this work, the ability to form biofilms on the surface of sterile coverslips by one clinical isolate of A. baumannii was studied. Sessile cells which adhered to coverslips after being immersed in a nutrient-deficient mineral medium were observed by epifluorescence and scanning electron microscopy at various times of incubation. A rapid increase in the number of sessile cells in young biofilms, followed by a slower increase of such cells was found. At 48 h biofilms were clearly visible and an amorphous material similar to the exopolysaccharide described in some other bacteria covered sessile cells was evident. Biofilm formation by A. baumannii probably favours its maintenance on solid surfaces in the hospital environment and protects the micro-organism against some antibacterial factors.

Acinetobacter↗

[The use of human gamma globulin in the treatment of common variable immunodeficiency].

BACKGROUND: The indication for treatment with human gammaglobulin in patients with primary hypogammaglobulinemia is well established. Nonetheless, there are no uniform criteria with regard to dose, periodicity and route of administration. METHODS: Twenty-seven patients with common variable immunodeficiency (CVI) who received i.m. or i.v. treatment with gammaglobulin were studied, evaluating the secondary effects, stable levels of IgG achieved, control of symptomatology, clinical evolution and the need for adjuvant therapies. RESULTS: Intravenous administration was more effective than intramuscular administration to achieve higher total IgG serum levels (5.2 +/- 1.2 vs 3.5 +/- 1.6 g/l; p = 0.07) in a shorter period of time (2.1 +/- 1.6 months vs 6.3 +/- 2.8 months; p < 0.01) and with new few secondary effects. The dose and periodicity of the treatment was individualized in each patient on the basis of the needs of consumption and the speed of metabolism of the gammaglobulin, with patients with chronic bronchial suppuration and diarrhea being those requiring the greatest doses (p < 0.0001) and a short interdosis time interval (19.2 +/- 3.1 vs 23.6 +/- 3.6 days; p = 0.01). Treatment with human gammaglobulin allowed the control of recurrent bacterial infection; however, adjuvant treatment with respiratory physical measures and antibiotics were required in patients with chronic bronchial suppuration to avoid progressive alteration of respiratory function. CONCLUSIONS: The administration of human gammaglobulin at adequate doses and frequency is effective to control infection, avoid the development of chronic bronchial disease, alteration of pulmonary function and the appearance of other complications. Intravenous route is safer and produces fewer secondary effects than intramuscular administration with the doses and period of the treatment requiring individualization for each patient.

Adolescent↗

Long-term survival in advanced ovarian cancer after cytoreduction and chemotherapy treatment.

Ninety-one patients with untreated epithelial ovarian cancer, stages III and IV, were treated according to a therapeutic protocol including cytoreductive surgery whenever possible, chemotherapy with CAP (cyclophosphamide, doxorubicin, and cisplatin) and second-look laparotomy for those patients achieving a clinical remission. Optimal cytoreductive surgery (residual tumor < 2 cm) was not performed in 66 patients (72.5%). A negative second-look laparotomy demonstrated a pathological complete remission in 26 patients (28.5%). After a median follow-up of 80 months, the disease-free survival is 19.7% (18 of 91 patients). Median survival was greater in optimal cytoreductive surgery patients (47 months) than in the rest of the patients (22 months) (P = 0.0000). Survival was also better in pathological complete remission patients (46 months) than in partial remission (PR) or no response patients (22 months) (P = 0.0001). Optimal secondary cytoreductive surgery was possible in 11 patients in PR after chemotherapy. Survival in this group was similar to that of pathological complete remission cases. Currently, 53% of patients with initial residual tumor < 2 cm and complete response at second-look remain free of disease. In a multivariate analysis, residual tumor > 2 cm and stage IV disease were the most significant prognostic factors. The same analysis indicates that response to chemotherapy at second laparotomy is not an independent prognostic factor. In conclusion, our study indicates that the two most important prognostic factors in advanced ovarian carcinoma are the extent of the initial surgery and stage.

Adult↗

[Evaluation of replacement therapy in emphysema caused by alpha 1-antitrypsin deficiency].

Assessment of alpha 1-antitrypsin replacement therapy (AAT) for emphysema. Patient characteristics were analyzed along with the possible side effects of the treatment and its efficacy in maintaining appropriate AAT blood levels. Lung function changes were also studied. The treatment protocol began with 4 weekly intravenous doses of 60 mg/kg AAT (Prolastin) and continued with monthly doses of 240 mg/kg. AAT serum levels were measured before each dose. Every 6 months pulmonary function tests (spirometry, plethysmography and CO transfer) were performed. Thirteen patients (mean age 46 yr) have been studied since 1988. Mean initial FEV1 was 0.79 l. Over 250 doses have been infused with no significant side effects reported. AAT levels before treatment in 3 patients were lower than that considered protective (50 mg/dl). Function tests results indicated stabilization of spirometric values in most cases. Diagnosis of AAT deficiency is delayed considerably, meaning that significant functional deterioration takes place before replacement therapy begins. No side effects of treatment have been observed. Until an appropriate interval between doses has been established, each patient's AAT levels must be monitored.

Adult↗

Scintigraphy, angiography and computed tomography in unilateral hyperlucent lung due to obliterative bronchiolitis.

This study examines the value of different imaging methods in assessing the anatomic structures of unilateral hyperlucent lung due to obliterative bronchiolitis. We studied 9 patients, 5 males and 4 females, suffering from UHL (mean age 49 years). Ventilation-perfusion scan (VPS) and computed tomography (CT) of the chest were performed in all, and conventional angiography or digital substraction angiography (DSA) in 7 patients. The VPS showed the characteristic pattern of a matched ventilation-perfusion defect and considerable air trapping during the washout phase. Conventional angiography and DSA displayed a smaller pulmonary artery on the affected side, with a poor peripheral vasculature. CT displayed a loss of lung volume in all cases, with diminished mean attenuation values, a markedly diminished vasculature and integrity of the main airways. In contrast to other imaging modalities, CT imaged bronchiectasis, which was the cause of the patients' clinical symptoms of bronchorrhea and hemoptysis. We conclude that CT of the chest is the most valuable imaging method for evaluating unilateral hyperlucent lung, particularly in symptomatic patients.

Adult↗

Terminal deletion of 6p: report of a new case.

The authors report the case of a girl with psychomotor and growth retardation, ventricular septal defect, patent ductus arteriosus, bulging forehead, prominent philtrum, low nasal bridge, inner epicanthal folds, strabismus, miosis, low set ears with prominence of antihelices, short neck, single transverse crease and camptodactyly of the fourth finger in both hands. G-banded chromosomal analysis revealed a 46, XX, del (6) (p23) chromosome constitution. A review of the phenotypes of the patients known with this chromosome deletion is presented.

Abnormalities, Multiple↗

[Treatment of respiratory insufficiency secondary to vocal cord bilateral paralysis with continuous positive pressure].

Vocal cord paralysis can produce extrathoracic airway obstruction with severe respiratory failure, post-surgical traumatism being the most frequent. Definitive treatment can require aritenoidectomy. For emergency treatment tracheal intubation of tracheotomy are frequently needed. We report a patient with acute post-surgical upper airway obstruction successfully treated with CPAP application through nasal mask. A 29 year-old female showed stridor and retraction of the supraclavicular, intercostal and epigastric region following an uncomplicated tracheal extubation immediately after surgery (radical thyroidectomy with nodal dissection). Pulsosaturometry showed O2 desaturation despite high flow O2 administration. She received intravenous steroids and O2 through intermittent positive pressure by nasal mask (manual resuscitator) increasing SpO2 to 90%. Laringoscopy showed both vocal cords fixed at medium line. CPAP through a nasal mask was initiated with a 5 cm H2O pressure and high FIO2. Immediately afterwards, dyspnea, stridor, supraclavicular retraction and respiratory accessory muscles use disappeared. Heart rate decreased (120 to 92 x min.) and SpO2 increased to 99%. Arterial blood gases did not show hypercapnia. Dyspnea and physical signs of upper airway obstruction appeared immediately after interrupting CPAP application, with a marked decrease in SpO2. So the mask was reinstalled keeping the same pressure level during 18 hs. The procedure was well tolerated. There were no local or hemodynamic complications. CPAP was progressively discontinued.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Plasma bioavailability of 2 long-acting oxytetracycline formulations in the pig.

Two commercially available long-acting oxytetracycline (OTC-LA) formulations were administered by intramuscular injection in 2 groups of 10 clinically healthy pigs at the recommended dose level of 20 mg/kg. Plasma concentrations were analysed by high-performance liquid chromatography (HPLC) of a period of 0 to 84 h. The limit of quantification of the assay was 0.125 microgram/ml. The comparison of the CMAX did not reveal any significant differences (4.45 +/- 1.30 and 4.40 +/- 0.9 micrograms/ml). The results were similar for the TMAX (3.60 +/- 1.58 and 4.00 +/- 2.67 h). The areas under the curve were also similar. The AUC0-84 h results were respectively 92.8 +/- 14.1 and 96.3 +/- 11.3 mg.h/l and the AUC0-infinity results were 99.5 +/- 14.7 and 106.7 +/- 15.4 mg.h/l. No significant difference was found. This may be considered as a preliminary study to demonstrate the bioequivalence of the 2 preparations. On the basis of the statistical analysis of the results obtained, a cross-over study using 2 groups of 20 animals is theoretically necessary for such a demonstration.

Animals↗