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Biomedical subjects

M Lima

Publications and source records attributed to M Lima.

At least 37 records · Page 2Linked to original sources

Immunophenotypic aberrations, DNA content, and cell cycle analysis of plasma cells in patients with myeloma and monoclonal gammopathies.

We describe the immunophenotypic and gross DNA defects in 55 patients with myeloma and 50 patients with monoclonal gammopathy and review the literature on this subject (MedLine, 1994-2000). Our data confirmed previous reports indicating that in myeloma nearly all marrow plasma cells are abnormal (98.7 +/- 8.1%). In monoclonal gammopathy the fraction of abnormal plasma cells was 35.0 +/- 32.8%. In both myeloma and monoclonal gammopathy, the most frequent aberrant phenotypic features consisted of absence of expression of CD19, strong expression of CD56, and decreased intensity of expression of CD38; aberrant expression of CD10, CD20, CD22, or CD28 was observed in less than one-third of myeloma cases. The vast majority of cases had two or more phenotypic aberrations. In the DNA studies, 7% of myeloma cases were biclonal and 93% of cases were monoclonal. In those studies with only one plasma cell mitotic cycle, 37% had normal DNA content and 63% were aneuploid (hyperploid, 61%; hypoploid, 2%). The mean percentages of plasma cells in S- and G2M phases were 4.9 +/- 8.5 and 4.4 +/- 6.9%, respectively. Thirty-eight percent of cases had more than 3% of plasma cells in S phase. In monoclonal gammopathy, the DNA index of abnormal plasma cells ranged from 0.89 to 1.30 and the percentage of diploid (31%) and aneuploid (69%) cases was not different from the results found in myeloma. The differences in percentage of abnormal plasma cells in S- (7.4 +/- 8.6%) and G2M-phases (2.4 +/- 1.7%) in patients with monoclonal gammopathy were not statistically significant.

Adult↗

Recurrent posttraumatic urethrovaginal fistula: a new application for ASTRA.

The authors describe the results of an application of the surgical technique called ASTRA (anterior sagittal transrectal approach) in the case of a 16-year-old girl with a posttraumatic urethrovaginal fistula. The young girl with a posttraumatic urethrovaginal fistula had recurrence after 4 surgical attempts at closure performed by the vaginal approach before it was definitively corrected with the ASTRA. Two years after the ASTRA operation the patient is well with a complete restitution of function confirmed by a voiding cystourethrogram and urodynamic and rectal manometric tests. The successful recovery confirmed that ASTRA may be an excellent method of treating the developmental or acquired anomalies of the perineal region.

Adolescent↗

Cardiocirculatory changes during videolaparoscopy in children: an echocardiographic study.

We examined cardiovascular changes associated with intra-abdominal insufflation in 20 children (mean age 6.1+/-4.7 years, ASA physical status I or II) undergoing laparoscopic surgery with general anaesthesia using echocardiography with a transthoracic approach. Intra-abdominal pressure never exceeded 10 mmHg. Systolic blood pressure, diastolic blood pressure, endtidal CO2, peak, and mean airway pressure increased during intra-abdominal insufflation (P < 0.001). Pneumoperitoneum was associated with increases (P<0.001) in left ventricular enddiastolic volume, left ventricular end-systolic volume and left ventricular endsystolic meridional wall stress. In addition, before, during and after intra-abdominal insufflation, left ventricular fractional shortening and left ventricular ejection fraction, underwent slight, insignificant changes. Pneumoperitoneum in children has a major impact on cardiac volumes and function, mainly through the effect on ventricular load conditions. The sharp increase in intra-abdominal pressure affects both preload and afterload, while systolic cardiac performance remains unchanged.

Anesthesia, General↗

Laparoscopic repair of Morgagni-Larrey hernia in a child.

Primary laparoscopic repair of Morgagni-Larrey hernia has been described in adult patients but not in children. This is the first report of primary laparoscopic correction in the pediatric age group without using a prosthesis. A Morgagni-Larrey hernia was found incidentally in a 3-year-old-girl. Laparoscopic correction of the defect was performed. After 6 months the patient is doing well. The chest radiograph shows complete resolution of the hernia. The laparoscopic approach allowed repair the hernia with minimal invasiveness. Laparoscopic correction is not difficult except for those hernias in which dense adhesions are present.

Child, Preschool↗

Diurnal blood pressure variation in progressive autonomic failure.

To investigate the role of the autonomic nervous system (ANS) in the generation of the circadian blood pressure (BP) variation, the degree of impairment of the ANS was related to the results of ambulatory BP recordings in 212 patients with progressive autonomic failure due to familial amyloid polyneuropathy. On the basis of BP and/or heart rate (HR) responses to the Valsalva maneuver, 60 degrees head-up tilting, deep-breathing tests, and plasma norepinephrine levels, 4 groups of patients were distinguished. In all patients and in 38 age-matched control subjects, ambulatory BP was monitored. Patients of group I (n=40, aged 32+/-3 y), with no evidence yet of impairment of their ANS, had circadian BP and HR variations indistinguishable from controls. Patients of group II (n=41, aged 34+/-5 y) had a variable degree of impairment of their parasympathetic ANS, but their sympathetic ANS was still intact. Twenty-four-hour HR was higher in these patients than in controls (88+/-11 versus 78+/-7 bpm, P<0.01). Their circadian HR variation was maintained, but their circadian BP variation was diminished (10+/-6/11+/-4 versus 17+/-6/16+/-4 mm Hg in controls, P<0.01) because of an attenuation of the nocturnal BP decline. Patients of group III (n=69, aged 36+/-6 y), with parasympathetic failure and intermediate sympathetic dysfunction, had a blunted diurnal BP variation, whereas patients of group IV (n=62, aged 38+/-6 y), with parasympathetic failure and severe sympathetic dysfunction, had an absent diurnal BP variation. In patients of groups III and IV, a decrease in daytime BP accounted for the blunted circadian BP variation. This extensive study in progressive autonomic failure confirms the important role of the ANS in the generation of circadian BP variation. For the maintenance of a normal circadian BP pattern, not only an intact sympathetic but also an intact afferent parasympathetic ANS is a prerequisite.

Adult↗

Production and characterization of monoclonal antibodies to Brazilian isolates of bovine viral diarrhea virus.

Three Brazilian isolates of bovine viral diarrhea virus (BVDV), antigenically distinct from the standard North American isolates, were selected to immunize BALB/c mice in order to obtain hybridoma cells secreting anti-BVDV monoclonal antibodies (mAbs). Two hybridoma clones secreting mAbs, reacting specifically with BVDV-infected cells (mAbs 3.1C4 and 6.F11), were selected after five fusions and screening of 1001 hypoxanthine-aminopterin-thymidine-resistant clones. These mAbs reacted in an indirect fluorescent antibody (IFA) assay with all 39 South and North American BVDV field isolates and reference strains available in our laboratory, yet failed to recognize other pestiviruses, namely the hog cholera virus. The mAbs reacted at dilutions up to 1:25,600 (ascitic fluid) and 1:100 (hybridoma culture supernatant) in IFA and immunoperoxidase (IPX) staining of BVDV-infected cells but only mAb 3.1C4 neutralized virus infectivity. Furthermore, both mAbs failed to recognize BVDV proteins by IPX in formalin-fixed paraffin-embedded tissues and following SDS-PAGE and immunoblot analysis of virus-infected cells, suggesting they are probably directed to conformational-type epitopes. The protein specificity of these mAbs was then determined by IFA staining of CV-1 cells transiently expressing each of the BVDV proteins: mAb 3. 1C4 reacted with the structural protein E2/gp53 and mAb 6.F11 reacted with the structural protein E1/gp25. Both mAbs were shown to be of the IgG2a isotype. To our knowledge, these are the first mAbs produced against South American BVDV isolates and will certainly be useful for research and diagnostic purposes.

Animals↗

Recurrent posttraumatic urethro-vaginal fistula: a new application for ASTRA.

The authors describe the results of an application of the surgical technique called ASTRA (anterior sagittal transrectal approach) in a 16-year-old girl with recurrent urethro-vaginal fistula. The young girl had a posttraumatic urethro-vaginal fistula. It recurred after 4 operations by a direct vaginal approach before definitive correction with the ASTRA. Three years after the operation the patient has remained well with complete healing and no fistula recurrence confirmed by a voiding cystourethrogram and urodynamic and rectal manometric tests. This report suggests that ASTRA is a useful method of treating acquired or developmental anomalies of the perineal region.

Adolescent↗

Influence of heavy agricultural work during pregnancy on birthweight in northeast Brazil.

BACKGROUND: Women in developing countries often continue their agricultural work during late pregnancy. Whether this adversely affects birthweight is not clear from previous studies as few controlled for confounding factors. This study seeks to clarify this issue. METHODS: This retrospective cohort study investigated 958 low-income women and their singleton newborn babies residing in a region of Northeast Brazil dependent on sugar-cane production. Women were recruited at maternity centres, when attending for delivery, and were allocated to one of two groups according to their exposure to heavy agricultural labour for at least 3 months during the second and third trimesters of pregnancy (n = 250), or to household activities only (n = 708). RESULTS: The mean birthweight of infants born to women who worked in agriculture during 9 months of pregnancy was 190 g lower than that of the non-exposed group (P = 0.02). After controlling for confounding factors, the adjusted effect was 117 g (P = 0.05). Heavy agricultural work for 6, 7 or 8 months had no significant effect. CONCLUSIONS: These findings suggest that working throughout pregnancy significantly reduces birthweight in this low-income population.

Adolescent↗

Causes of death in Machado-Joseph disease: a case-control study in the Azores (Portugal).

BACKGROUND: Machado-Joseph disease (MJD) is an autosomal dominant cerebellar ataxia of adult onset with a high prevalence in the islands of Azores (Portugal). The genetic epidemiological studies presently under way in these islands are based on the genealogical reconstruction of the affected families, thus partially depending on the reference of patients using family history. A considerable effort has been made to obtain genealogies that are as complete as possible, making use of different types of data. The utility of the death causes contained in the death registers of the patients with MJD was determined in this study. OBJECTIVES: To estimate the extent to which the cause of death reported in the death register can confirm other reports of an individual's status for the disease (ie, oral information), and to determine the accuracy of the death certificates in listing MJD in patients whose disease was clinically diagnosed. DESIGN: Case-control study. METHODS: The death registers of 113 patients with MJD (82 whose disease was identified by history and 31 whose disease was clinically diagnosed) were examined and compared with those of controls matched by sex and date and place of death. RESULTS: There were significant differences in the causes of death between cases and controls, both for those whose disease was identified by history (chi(2) = 51.69, P < .001) and for those whose disease was identified by examination (chi(2) = 27.78, P = .004). However, the cause of death was in accord with the presence of the disease in only 40% of the cases reported as being identified only by family history. In the cases in which the disease was clinically diagnosed, only nearly 38% of the registers provided reliable information as to MJD being the direct cause of death. CONCLUSIONS: The fact that only nearly 40% of the patients with clinically confirmed MJD had a cause of death compatible with MJD precludes the use of cause of death as a means of identifying affected individuals in the Azorean MJD pedigrees.

Adult↗

Multicystic kidney joined to the homolateral testis by an ectopic vas deferens: embryological hypothesis for a new description.

Ectopic location of the vas deferens is a rare congenital anomaly with multiple variations. The frequent association with ano-rectal abnormalities and hypospadias is well known. We report two cases of a newborn and a 3-year-old child with ectopic vas deferens coupled with a left multicystic kidney without any ureteral structure. Furthermore, the latter had a contralateral vesicoureteral reflux and anal agenesis with recto-bulbar fistula. Our attempt with this report is to explain the development of such anomalous deferential outlet into the multicystic kidney, according to the most qualified theory of the Wolffian duct embryology.

Child, Preschool↗

Pure red cell aplasia associated to clonal CD8+ T-cell large granular lymphocytosis: dependence on cyclosporin A therapy.

This case report details a single patient with pure red cell aplasia (PRCA) associated with clonal CD3+, TCRalphabeta+, TCR-Vbeta8+, CD8+, CD57+ large granular lymphocytosis whose anaemia did not respond to conventional immunosuppressive therapy but did respond to cyclosporin A (CsA). The patient has become dependent on CsA for 7 years in order to control anaemia due to associated PRCA.

Aged↗

Origins of a mutation: population genetics of Machado-Joseph disease in the Azores (Portugal).

Machado-Joseph disease (MJD) is an autosomal dominant neurodegenerative disorder of adult onset. In the islands of the Azores (Portugal), MJD reaches the highest prevalence reported worldwide. It has been postulated that it is highly represented in the Azorean population as a result of a founder effect. To test this hypothesis, we reconstructed the ascending genealogies of the 32 Azorean families presently identified as harboring the disease (103 patients), using parish records as the main source of data. These patients were originally from the islands of São Miguel, Terceira, Graciosa, and Flores. The genealogies of the two main Azorean American families (Machado and Joseph) were also reconstructed. To identify the links between the MJD families, we calculated the kinship coefficient between the proponents of these genealogies. The family from Terceira was linked to three different MJD families from Flores through common ancestors. No kinship was observed between the MJD families from São Miguel and families from any other island. Links between the two Azorean American families and Azorean MJD families were found. The founders present in more than one ascendance were identified. Their chronological and geographic distribution indicates that more than one MJD mutation was introduced in the Azores, probably by settlers coming from the Portuguese mainland. The molecular evidence to date corroborates these results, because two distinct haplotypes have been established, one on the island of São Miguel and the other on Flores. Therefore molecular biology studies confirm the accuracy of the conclusions drawn from the genealogical evidence supporting the absence of a founder effect for MJD in the Azorean population.

Adult↗

BCL-2 oncoprotein (p26) in splenic lymphoma with villous lymphocytes: a comparative study with other chronic B-cell disorders.

We compared the expression of the BCL-2 oncoprotein (p26) on B cells from 24 patients with splenic lymphoma with circulating villous lymphocytes (SLVL) with that observed on normal, mature B lymphocytes and on neoplastic B cells from 91 patients with other chronic B-cell malignancies. SLVL B cells showed levels of p26 intermediate between those found on normal B lymphocytes and on neoplastic B cells from patients with other chronic B-cell lymphoproliferative disorders.

B-Lymphocytes↗

Decreased expression of bcl-2 (p26) in CD8(+) lymphocytes of patients with T-cell lymphoproliferative disorders of large granular lymphocytes.

The bcl-2 oncogene has been involved in the genesis of various B-cell neoplasms by means of encoding for p26, an apoptosis suppressor oncoprotein. The expression of p26 in lymphoproliferative disorders of large granular lymphocytes (LDLGL), a group of diseases whose mechanism leading to lymphocyte expansion is not yet clear, was not previously characterized. In order to further understand the biology of LDLGL, we compared the expression of p26 in CD8(+) lymphocytes of patients with CD3(+) LDLGL with that observed in normal individuals, patients with viral infection and patients with CD4(+) lymphoid neoplasms. We observed that upregulation of bcl-2 expression is not involved in the genesis of lymphocyte expansion in CD3(+) LDLGL. By contrast, when compared to normal peripheral blood counterparts, CD8(+, bright) lymphocytes of patients with LDLGL express low levels of p26 whereas CD8(+, dim) lymphocytes express normal or only slightly reduced levels of this oncoprotein. A similar pattern of expression of p26 was found in situations in which CD8(+) lymphocytes represent reactive activated cells.

Adult↗

A comprehensive knowledge-based system for laboratory hematology.

The Coulter FACULTY knowledge-based systems, Professor Petrushka for peripheral blood interpretation, Professor Fidelio for flow cytometry immunophenotyping and Professor Belmonte for bone marrow reporting, have been installed in several hospitals in Spain, Portugal and the United Kingdom. In Spain and Portugal, the systems are part of the IZASA-Coulter CITOTECA workstation, which includes a video camera for capturing microscopic images and a networkable laboratory information system supporting color reports. At the Royal Hospitals Trust (St. Bartholomew's Hospital and The Royal London Hospital, London, UK), networked workstations are available and the system is used daily to generate bone marrow reports in the hematology laboratories. There have been considerable benefits from adopting Coulter FACULTY for bone marrow reporting, including faster turnaround time, improved quality of the reports and cost savings.

Artificial Intelligence↗