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Biomedical subjects

H Rocha

Publications and source records attributed to H Rocha.

At least 19 recordsLinked to original sources

Cyclophosphamide in the treatment of focal segmental glomerulosclerosis.

Prednisone is the initial treatment of primary focal segmental glomerulosclerosis. However, when immunosuppressive agents in combination with steroids are used in the treatment of prednisone-dependent and prednisone-resistant patients the remission rate is variable. We report a long-term trial using cyclophosphamide (2.0 to 3.0 mg/kg body weight for 12 weeks) in combination with prednisone (1.0 to 2.0 mg/kg body weight), as compared with prednisone alone for the treatment of prednisone-resistant and frequently relapsing nephrotic syndrome and focal segmental glomerulosclerosis. Fifty-four patients (34 males and 20 females) with a diagnosis of idiopathic nephrotic syndrome and focal segmental glomerulosclerosis, followed-up for an average of 86.1 +/- 82.4 months, were evaluated. Complete remission occurred in 20.4% and partial remission in 14.8% of the patients treated with steroids and in 26.7 and 20.0% of the patients treated with cyclophosphamide + prednisone, respectively. Of the 24 prednisone-resistant patients treated with steroids in combination with cyclophosphamide, 33.3% obtained a complete/partial response. At the time of final evaluation, 25% of the patients treated with prednisone and 10.0% of those treated with prednisone in combination with cyclophosphamide had reached end-stage renal disease. Persistent nephrotic syndrome and progressive renal insufficiency were more frequently observed among the patients treated with prednisone alone (50.0 vs 33.3% and 33.3 vs 16.7%, respectively). The treatments were well tolerated and no patient experienced adverse reactions requiring discontinuation of medications. Although open-label and non-randomized, the present trial showed that cyclophosphamide is a reasonable choice for the treatment of primary focal segmental glomerulosclerosis and prednisone-resistant nephrotic syndrome.

Adolescent↗

Distribution and genetic diversity of tomato-infecting begomoviruses in Brazil.

Tomato-infecting begomoviruses have been reported throughout Brazil since the introduction of the B biotype of Bemisia tabaci. Here, we report a large scale survey on the distribution and genetic diversity of tomato-infecting begomoviruses. Tomato samples with typical begomovirus symptoms were collected in seven different states, comprising the major tomato growing areas of the country. Viruses were detected by polymerase chain reaction (PCR) using universal primers for the genus Begomovirus. PCR-amplified fragments were cloned and sequenced. Based on sequence comparisons and phylogenetic analyses, at least seven previously undescribed species of begomoviruses were found. Four of the new viruses were found exclusively in the Southeastern states, two exclusively in the Northeastern states, and one was found in both regions. Sequence comparisons reveal strong evidence of recombination among the Brazilian begomoviruses. Together, the results indicate the existence of a high degree of pre-existing genetic diversity among tomato-infecting begomoviruses in Brazil and suggest that these viruses have emerged after being transferred from natural hosts to tomatoes, due to the introduction into Brazil of a novel polyfagous biotype of the whitefly vector.

Base Sequence↗

Race and glomerulonephritis in patients with and without hepatosplenic Schistosomiasis mansoni.

BACKGROUND/AIMS: United States investigators have shown evidence of higher susceptibility to focal segmental glomerulosclerosis (FSGS) in blacks than in whites. This association between race and FSGS has not been assessed outside the US. The present study assesses the association between race and type of glomerulonephritis in a sample of Brazilian patients, taking into account the presence of the hepatosplenic form of Schistosomiasis mansoni (HSM). METHODS: Eighty patients with focal segmental glomerulosclerosis (FSGS) were compared to 50 with membranoproliferative glomerulonephritis (MPGN). The association between race (i.e. black versus white) and type of glomerulonephritis was adjusted for age, gender and HSM by logistic regression. RESULTS: Blacks were more likely than whites to have FSGS (as compared to MPGN), both among patients with HSM (odds ratio (OR) = 2.67; 95% confidence interval (CI) = 0.81 - 8.81) and without HSM (OR = 2.19; 95% CI = 0.79 - 6.05). After adjustment for age, gender and HSM, the odds of FSGS remained significantly greater for blacks (OR = 2.49; 95% CI = 1.05 - 5.95). CONCLUSION: The increased likelihood of FSGS in Brazilian blacks is consistent with findings from US patients. The association between race and type of glomerulonephritis was similar between patients with and without HSM. Future investigations should focus on the mediators factors that might explain these findings.

Adolescent↗

Community acquired pneumonia among pediatric outpatients in Salvador, Northeast Brazil, with emphasis on the role of pneumococcus.

Pneumonia is one of the leading causes of hospitalization and death among children in developing countries, and mortality due to pneumonia has been associated with S. pneumoniae infection. This investigation was designed to describe the antimicrobial susceptibility and serotype patterns of pneumococcal strains recovered from the blood of children with community-acquired pneumonia (CAP) and to assess the clinical findings of pneumococcal bacteremic patients with pneumonia. In a 26 month prospective study, blood cultures were obtained as often as possible from children (<16 years of age) diagnosed with CAP in two emergency rooms. Antimicrobial drug susceptibility tests and serotyping were performed when pneumococcus was identified. We studied 3,431 cases and cultured blood samples from 65.5% of those. Pneumococcus was recovered from 0.8% of the blood samples. The differences in age, somnolence, wheezing and hospitalization among children with and without pneumococcal bacteremia were statistically significant. Pneumococcal bacteremia was age-related (mean 1.63 +/- 1.55; median 0.92) and associated with somnolence and hospitalization among children with CAP. One strain was recovered from pleural fluid. Penicillin resistance was detected in 21.0% (4/19) of the strains at an intermediate level, whereas 63.0% of the strains were resistant to trimethoprim-sulfamethoxazole. The most common serotypes were 14 and 6B, and these serotypes included the resistant strains. Eight of our 18 isolates from blood were of types included in the heptavalent conjugate pneumococcal vaccine, recently licensed in the USA.

Adolescent↗

Primary focal segmental glomerulosclerosis in children: prognostic factors.

To study the clinical course and the predictors of outcome in children and adolescents, 39 patients with nephrotic syndrome and primary focal segmental glomerulosclerosis (FSGS) were followed for a mean of 84.6 months. Thirty-six patients were treated with prednisone, either alone or in conjunction with cyclophosphamide. The clinical course was one of sustained remission in 4 patients, frequent relapse in 13, persistent nonnephrotic proteinuria in 5, and persistent nephrotic syndrome in 17; 2 patients had stable renal failure and 8 had progressive renal failure, 5 of them evolving to end-stage renal failure (ESRF). Resistance to prednisone was recorded in 76.6% of patients. The use of cyclophosphamide plus prednisone was of benefit in 42.8% of patients; 22.2% of the prednisone-resistant patients achieved remission of the nephrotic syndrome. A Kaplan-Meier analysis revealed a survival rate of 92% after 5 years, 86% after 10 years, and 76% after 15 years. Using both univariate and multivariate analysis, persistent nephrotic syndrome was associated with progression to ESRF and the remission of proteinuria with maintenance of renal function. As the outcome of the nephrotic syndrome in FSGS is significantly improved by remission of proteinuria, it is conceivable that immunosuppressive medication may be used in conjunction with prednisone in patients with steroid resistance.

Adolescent↗

Transcanal labyrinthectomy in childhood: report of a case.

Vertigo in children is caused by several factors. One such factor is viruses (e.g., mumps, measles, and rubeola) afflicting the inner ear by various mechanisms. In the majority of cases, affected patients improve with clinical therapy and vestibular rehabilitation. We present the case of a child with vertigo as a sequela of mumps virus infection that did not improve with clinical treatment and vestibular rehabilitation. Consequently, the child was submitted to a transcanal labyrinthectomy followed by vestibular rehabilitation, with significant postprocedure improvement of symptoms. We discuss surgical indications, the technique used in this case, and the importance of vestibular rehabilitation in the postoperative period.

Child↗

Clinical and molecular studies in three Portuguese mtDNA T8993G families.

The T8993G mutation in the mitochondrial DNA adenosine triphosphatase 6 gene represents an important cause of maternally inherited Leigh's syndrome. Reported are the clinical findings and mutational loads in three Portuguese T8993G pedigrees. Polymerase chain reaction-restriction fragment length polymorphism analyses demonstrated the T8993G mutation in a high percentage of tissues from all patients (97% +/- 2.3%), but it was less abundant in the blood from 14 maternal relatives. The disease progressed severely in the probands but did not have the fatal course reported by others. To test whether this prolonged course was related to the presence of a specific, disease-associated haplogroup the origin of the mutational event in Portugal was traced. Haplotype investigation revealed an independent occurrence of the mutation in the three probands. These analyses represent the first molecular characterization of Portuguese patients with Leigh's syndrome.

Adenosine Triphosphatases↗

Clonally related penicillin-nonsusceptible Streptococcus pneumoniae serotype 14 from cases of meningitis in Salvador, Brazil.

Active hospital-based surveillance in the city of Salvador, Brazil, from December 1995 through October 1998, identified 221 patients with confirmed pneumococcal meningitis. Of these 221 patients, 29 (13%) had isolates with intermediate-level resistance to penicillin. Infection with these penicillin-nonsusceptible isolates was significantly associated with age of <2 years (P<.0019), previous antibiotic use (P<.0006), and coresistance to trimethoprim-sulfamethoxazole (P<.0000). Serotype 14 was the most prevalent serotype (55.2%) of penicillin-nonsusceptible isolates. Strain typing by repetitive element BOX polymerase chain reaction (PCR) analysis showed that penicillin-nonsusceptible serotype 14 isolates had closely related BOX PCR patterns, whereas penicillin-susceptible serotype 14 isolates each had distinct, unrelated patterns. Penicillin-nonsusceptible serotype 14 isolates from Salvador and other Brazilian cities had similar BOX PCR patterns. These observations indicate that in Brazil a large proportion of cases of penicillin-nonsusceptible pneumococcal meningitis appear to be caused by a closely related group of serotype 14 strains that may have disseminated to widely separate geographic areas.

Adolescent↗

Mucosal leishmaniasis: quantitative nasal cytology as a marker of disease activity and indicator of healing.

Mucosal leishmaniasis (ML) follow-up is based on subjective parameters. Using simplified quantitative cytology of nasal lavages (QNCs), we studied 20 ML patients, 10 patients with cutaneous leishmaniasis (CL), and 10 healthy subjects. Patients with ML were treated with antimony and followed up with otolaryngological examination plus QNCs for 6 months. At the first evaluation, the median total number of cells in ML patients (1,540,000) was greater than that in CL patients (215,000) or that in healthy subjects (250,000). Neutrophils were predominant in ML patients, in contrast to both sets of controls, in whom epithelial cells were more frequent. During treatment, we found a significant reduction in total nasal cell counts in ML patients who were cured, and encountered a switch in predominant cell type. The cytology of 2 patients who did not respond to antimony remained the same. It is therefore possible to detect nasal inflammation in ML patients through QNCs, which may indicate extension of mucosal involvement, providing an objective parameter to monitor therapy.

Adolescent↗

Missense mutation R1066C in the second transmembrane domain of CFTR causes a severe cystic fibrosis phenotype: study of 19 heterozygous and 2 homozygous patients.

We report the clinical features of 21 unrelated cystic fibrosis (CF) patients from Portugal and Spain, who carry the mutation R1066C in the CFTR gene. The current age of the patients was higher in the R1066C/any mutation group (P < 0.01), as compared to the deltaF508/deltaF508 group. Poor values for lung radiological involvement (Chrispin-Norman) and general status (Shwachman-Kulcycki) were observed in the R1066C/any mutation group (P < 0.005 and P < 0.0004). A slightly, but not significantly worse lung function was found in the R1066C/any mutation group when compared with the deltaF508/deltaF508 patients. No significant differences were detected regarding the age at diagnosis, sweat Cl-values, or percentiles of height and weight between the two groups. Neither were significant differences observed regarding sex, meconium ileus (4.7% vs. 11.1%), dehydration (10.5% vs. 14.7%), or pancreatic insufficiency (PI) (100% vs. 97.8%). The proportion of patients with lung colonization by bacterial pathogens was slightly, but not significantly higher in the R1066C/any mutation group (70.0%), as compared with the deltaF508/deltaF508 group (57.5%). Other clinical complications were significantly more frequent in the R1066C/any mutation patients(P < 0.02) than in the deltaF508/deltaF508 group. The two homozygous R1066C/R1066C patients died at the ages of 3 months and 7 years. The data presented in this study clearly demonstrate that the R1066C mutation is responsible for a severe phenotype similar to that observed in homozygous deltaF508 patients. The poor clinical scores and complications of patients with the R1066C mutation are probably related to their slightly longer survival.

Adolescent↗