Publications (13)17.01 Total impact
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Article: Diagnosis and management of congenital rectourethral fistula in a child with long tubular duplication of the colon and Klippel-Feil syndrome.
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ABSTRACT: We describe a unique association of congenital rectourethral fistula with long tubular duplication of the colon in a boy with Klippel-Feil syndrome and Sprengel deformity. He presented with a rectourethral fistula after surgical repair of a tubular duplication of the terminal ileum, colon, and proximal rectum. Preoperative identification of the fistula was challenging and was only achieved after cystoscopy with injection of methylene blue under pressure through Foley catheters placed into the anus and distal stoma of a colostomy. Surgical repair was performed through the posterior sagittal approach. The patient is doing well after 4 years of follow-up.Journal of Pediatric Surgery 11/2011; 46(11):2184-6. · 1.45 Impact Factor -
Article: Impact of antibiotic therapy on laboratory analysis of parapneumonic pleural fluid in children.
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ABSTRACT: The therapeutic management of parapneumonic pleural effusions (PPE) is controversial in children. Decision-making often relies on parameters such as gross appearance of pleural fluid and on bacteriologic and biochemical analyses. Our goal was to describe the laboratory profile of PPE in children and to assess the influence of previous administration of antibacterial agents on culture and biochemical results. This was a prospective study including children (age, 1 month to 16 years) with a diagnosis of PPE. Two groups were evaluated: children with or without antibiotic treatment up to 48 hours before analysis of pleural fluid. Results were analyzed using the χ(2) or Mann-Whitney test (α = .05). Odds ratio and 95% confidence intervals (95% CIs) were calculated, with control of previous antibiotic therapy using multivariate logistic regression analysis, to determine the risk of empyema associated with specific biochemical parameters. One hundred ten children were selected. Fifty percent had received antibiotics at least 48 hours before pleural fluid analysis. Differences were observed between the groups in terms of PPE gross appearance (P = .033) and identification of bacteriologic agent by culture or Gram stain (P = .023). Biochemical parameters (pH ≤7.1 and glucose ≤40 mg/dL) were associated with increased odds of receiving a more invasive treatment. For pH, the odds ratio was 9.614 (95% CI, 1.952-47.362; P = .005); and for glucose, 9.201 (95% CI, 1.333-63.496; P = .024). Previous use of antibacterial agents affected the bacteriologic analysis of pleural fluid in this pediatric sample admitted for PPE. However, it did not interfere significantly with biochemical parameters of pleural fluid.Journal of Pediatric Surgery 03/2011; 46(3):452-7. · 1.45 Impact Factor -
Article: Tumor necrosis factor alpha in experimental empyema thoracis.
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ABSTRACT: To determine serum and pleural concentrations of tumor necrosis factor alpha (TNF-α) in an experimental model of empyema induced by intrapleural inoculation of Staphylococcus aureus or Streptococcus pneumoniae. Wistar rats were inoculated with S. aureus (SA group, 17 animals) or S. pneumoniae (SP group, 30 animals). The presence of free fluid or pus in the pleural space was investigated. TNF-α levels >150 pg/ml (minimum detection limit) were determined in pleural fluid and blood. Histopathological examination of pleural tissue was performed to determine the severity of infection. Serum TNF-α was >150 pg/ml in nine SA versus 10 SP rats. In pleural fluid, TNF-α was >150 pg/ml in 11 SA versus 19 SP rats. Pleural and serum TNF-α concentrations were significantly different in the SP group (P = 0.035), but not in the SA group (P = 0.727). Pleural TNF-α was similar in both groups (P = 0.92), but serum TNF-α was significantly higher in SA (P = 0.03). Out of 17 SA animals, 1 (5.8%) did not develop empyema, versus 4 (13.3%) out of 30 SP animals. A mild inflammatory response was predominant in both groups, but the inflammatory process was significantly more severe in SP (P = 0.012). However, TNF-α levels were not associated with severity of the inflammatory response. We describe a simple and effective rat model of empyema. TNF-α levels above 150 pg/ml in the pleural fluid are useful to confirm empyema, but cannot predict the severity of the inflammatory response. TNF-α levels below 150 pg/ml are useful to rule out empyema.Pediatric Pulmonology 12/2010; 45(12):1201-4. · 2.53 Impact Factor -
Article: Surgical treatment for pediatric mediastinal neurogenic tumors.
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ABSTRACT: Mediastinal neurogenic tumors are relatively rare in children, and controversies persist about the best surgical approach and long-term survival. With ethics approval we reviewed the case notes of 43 consecutive children (22 boys) operated on for mediastinal neurogenic tumor in two different institutions from March 1998 to June 2009. The average age at diagnosis was 3 years (range, 0.6 to 4.7 years). Of the 43 operated tumors, 20 were neuroblastomas (47%), 13 were ganglioneuroblastomas (30%), and 10 were ganglioneuromas (23%). Only 6 (14%) of these tumors were discovered incidentally; the remaining 37 (86%) presented symptomatically: cough (30%), dyspnea (21%), wheezing (11.6%), neurologic spinal compression (7%), dancing eyes syndrome (7%), and Horner syndrome (7%). Tumor resection was undertaken by open operation in 38 cases (88%) and by thoracoscopy in 5 cases (12%). The children operated on by means of thoracoscopy had significantly smaller tumors (p = 0.01) and shorter duration of thoracic drain (p = 0.011) and hospitalization (p = 0.016) than those who were operated on by thoracotomy. There was not any surgical death. Postoperative complications occurred in 11 children (25.6%). Tumor recurrence occurred in 4 children (9.3%) operated on by open surgery, and there were 2 deaths of children with neuroblastoma. The overall survival was 95.4% in an average follow-up of 3.5 years (range, 0.7 to 4.4 years). Surgical resection of children with mediastinal neurogenic tumors treated at two international tertiary hospitals was safe, and the thoracoscopic approach was appropriate for small tumors. The long-term survival was higher when compared with those reported for other primary neurogenic tumor locations.The Annals of thoracic surgery 08/2010; 90(2):413-8. · 3.74 Impact Factor -
Article: Mucopolysaccharidosis I, II, and VI: Brief review and guidelines for treatment.
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ABSTRACT: Mucopolysaccharidoses (MPS) are rare genetic diseases caused by the deficiency of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG) breakdown pathway. This metabolic block leads to the accumulation of GAG in various organs and tissues of the affected patients, resulting in a multisystemic clinical picture, sometimes including cognitive impairment. Until the beginning of the XXI century, treatment was mainly supportive. Bone marrow transplantation improved the natural course of the disease in some types of MPS, but the morbidity and mortality restricted its use to selected cases. The identification of the genes involved, the new molecular biology tools and the availability of animal models made it possible to develop specific enzyme replacement therapies (ERT) for these diseases. At present, a great number of Brazilian medical centers from all regions of the country have experience with ERT for MPS I, II, and VI, acquired not only through patient treatment but also in clinical trials. Taking the three types of MPS together, over 200 patients have been treated with ERT in our country. This document summarizes the experience of the professionals involved, along with the data available in the international literature, bringing together and harmonizing the information available on the management of these severe and progressive diseases, thus disclosing new prospects for Brazilian patients affected by these conditions.Genetics and Molecular Biology 01/2010; 33(4):589-604. · 0.63 Impact Factor -
Article: Is mortality rate influenced by the site of involvement in neonates undergoing laparotomy for necrotizing enterocolitis?
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ABSTRACT: The aim of the study was to evaluate the effect of disease site on the mortality rate of newborns with necrotizing enterocolitis (NEC) undergoing exploratory laparotomy. The study used prospective cohort of 141 consecutive newborns with NEC who underwent laparotomy from November 1991 to December 2005. One hundred nineteen (84.4%) newborns were premature. Small for gestational age was observed in 57 (40.4%). Sixty-eight (48.2%) infants died. Bivariate analysis revealed that involvement of the jejunum and ileum was associated with high mortality rates (20 deaths; 76.9%; odds ratio [OR], 20; 95% confidence interval [CI], 4.6-96.3; P < .001) and that coinvolvement of the jejunum was associated with greater disease extent. After controlling for individual variables, logistic regression showed that the mortality associated with jejunum and ileum involvement (OR, 0.61; 95% CI, 0.06-6.14; P = .68) did not differ from that associated with large bowel involvement (OR, 2.91; 95% CI, 0.81-10.50; P = .10). When the jejunum was involved with the disease, the percentage of involvement of the entire intestine was 72%, and the mortality rate was 85.1%. When the other bowel segments were involved, the global percentage of disease extent ranged from 42% to 49%, and the mortality rate from 51.9% to 71.8%. After multivariable adjustment disease site, jejunal involvement appeared to be only a surrogate marker of disease extent. The NEC-related mortality in newborns undergoing laparotomy was not influenced by disease site (small or large bowel). However, jejunum coinvolvement was a marker of greater disease extent and therefore of poor prognosis.Journal of Pediatric Surgery 08/2009; 44(8):1534-9. · 1.45 Impact Factor -
Article: Thoracoscopy in children with complicated parapneumonic pleural effusion at the fibrinopurulent stage: a multi-institutional study.
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ABSTRACT: To determine the efficacy of thoracoscopy in the management of children with complicated parapneumonic pleural effusion at the fibrinopurulent stage. Retrospective study of 99 children submitted to thoracoscopy for the treatment of complicated parapneumonic pleural effusion at the fibrinopurulent stage between November of 1995 and July of 2005. The mean age was 2.6 years (range, 0.4-12 years), and 60% were males. Thoracoscopy was performed at three different hospitals following the same treatment algorithm. Thoracoscopy was effective for 87 children (88%). In 12 (12%), a second surgical procedure was required: another thoracoscopy (n = 6) or thoracotomy/thoracostomy (n = 6). Mean duration of chest tube drainage following successful thoracoscopy was 3 days vs. 10 days in patients submitted to a second procedure (p < 0.001). In all of the children, the pleural infection resolved after treatment. Thoracoscopy-related complications included air leak (30%), chest tube bleeding (12%), subcutaneous emphysema associated with trocar insertion (2%) and surgical wound infection (2%). None of the children required additional surgical procedures due to the complications. The effectiveness of thoracoscopy in children with parapneumonic pleural effusion at the fibrinopurulent stage was 88%. The procedure was safe, with a low rate of severe complications. Thoracoscopy should be the first-choice treatment for children with parapneumonic pleural effusion at the fibrinopurulent stage.Jornal brasileiro de pneumologia: publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia 08/2009; 35(7):660-8. -
Article: Surgical treatment of congenital pulmonary arteriovenous fistula in children.
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ABSTRACT: Congenital pulmonary arteriovenous fistulas are rare vascular malformations in which an abnormal connection develops between an artery and a vein in the lung. We report the case of a 5-year-old boy with a history of cyanosis since birth and cough for 2 days. Physical examination revealed cyanosis of oral mucosa and extremities, digital clubbing, and 82% O(2) saturation on room air. Chest radiograph showed a nodule in left lower lobe; echocardiogram was normal, but chest computed tomography showed 2 nodules in left lower lobe. Arteriography showed 2 large arteriovenous fistulas in left lower lobe. Patient underwent left lower lobectomy, and surgical outcome was excellent. At hospital discharge, mucosal color was normal and O(2) saturation was 96% on room air. Congenital pulmonary arteriovenous fistulas should be suspected in children with cyanosis but no cardiac malformations. Symptomatic congenital pulmonary arteriovenous fistulas should be treated with embolization when fistulas are small and multiple, or with lung resection when they are large and localized.Journal of Pediatric Surgery 07/2008; 43(7):1365-7. · 1.45 Impact Factor -
Article: Bronchoscopy and tracheotomy removal of bronchial foreign body.
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ABSTRACT: On rare occasions, endoscopic extraction of airway foreign bodies is not feasible or may be associated with large risk for the patient; in those situations, open surgery is indicated. The authors report a case of an 8-year-old boy presenting with an airway foreign body that was too large to be extracted through the subglottic region. After several attempts, extraction was accomplished through a tracheotomy with bronchoscopic control. After removal, the cervical opening was closed. Since the tracheal suture was firm and without air leaks, a tracheostomy cannula was not placed. The tracheal tube was removed after 3 days. Follow-up 1 month after the procedure showed that all respiratory symptoms had disappeared.Journal of Pediatric Surgery 09/2002; 37(8):1239-40. · 1.45 Impact Factor -
Article: [Surgical treatment of parapneumonic pleural effusion and its complications].
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ABSTRACT: Surgical treatment of parapneumonic pleural effusion in children is controversial. The type of intervention is based mainly on personal experience and on the small number of reported cases. This article aims at presenting a literature review and the authorś experience in the surgical management of parapneumonic pleural effusion in children. Data were searched in the Medline and Lilacs databases. Complicated parapneumonic effusion should be surgically drained if thoracentesis reveals the presence of pus, positive Gram stain or culture, or pH less than 7.0 or glucose less than 40 mg/dl. The surgical drainage depends on the stage of parapneumonic pleural effusion: at the acute stage closed thoracostomy drainage is enough; at the fibrinopurulent stage thoracoscopy is indicated; at organizational stage thoracotomy can be performed in children with stable anesthetic conditions, and open thoracostomy drainage should be used in patients in a poor state of health. Echography is very important to evaluate the staging of parapneumonic effusion. Surgical treatment of complicated parapneumonic effusion should be done as early as possible, and the kind of procedure depends on the stage of pleural effusion. In children with complicated parapneumonic effusion echography is very important to evaluate the staging of parapneumonic effusion.Jornal de Pediatria 78 Suppl 2:S161-70. · 1.01 Impact Factor -
Article: Surgical Treatment for Pediatric Mediastinal Neurogenic Tumors
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ABSTRACT: BackgroundMediastinal neurogenic tumors are relatively rare in children, and controversies persist about the best surgical approach and long-term survival.MethodsWith ethics approval we reviewed the case notes of 43 consecutive children (22 boys) operated on for mediastinal neurogenic tumor in two different institutions from March 1998 to June 2009.ResultsThe average age at diagnosis was 3 years (range, 0.6 to 4.7 years). Of the 43 operated tumors, 20 were neuroblastomas (47%), 13 were ganglioneuroblastomas (30%), and 10 were ganglioneuromas (23%). Only 6 (14%) of these tumors were discovered incidentally; the remaining 37 (86%) presented symptomatically: cough (30%), dyspnea (21%), wheezing (11.6%), neurologic spinal compression (7%), dancing eyes syndrome (7%), and Horner syndrome (7%). Tumor resection was undertaken by open operation in 38 cases (88%) and by thoracoscopy in 5 cases (12%). The children operated on by means of thoracoscopy had significantly smaller tumors (p = 0.01) and shorter duration of thoracic drain (p = 0.011) and hospitalization (p = 0.016) than those who were operated on by thoracotomy. There was not any surgical death. Postoperative complications occurred in 11 children (25.6%). Tumor recurrence occurred in 4 children (9.3%) operated on by open surgery, and there were 2 deaths of children with neuroblastoma. The overall survival was 95.4% in an average follow-up of 3.5 years (range, 0.7 to 4.4 years).ConclusionsSurgical resection of children with mediastinal neurogenic tumors treated at two international tertiary hospitals was safe, and the thoracoscopic approach was appropriate for small tumors. The long-term survival was higher when compared with those reported for other primary neurogenic tumor locations.The Annals of Thoracic Surgery. -
Article: [Enzyme replacement therapy for mucopolysaccharidoses I, II and VI: recommendations from a group of Brazilian F experts].
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ABSTRACT: Mucopolysaccharidoses (MPS) are rare genetic diseases caused by deficiency of specific lysosomal enzymes that affect catabolism of glycosaminoglycans (GAG). Accumulation of GAG in various organs and tissues in MPS patients results in a series of signs and symptoms, producing a multisystemic condition affecting bones and joints, the respiratory and cardiovascular systems and many other organs and tissues, including in some cases, cognitive performance. So far, eleven enzyme defects that cause seven different types of MPS have been identified. Before introduction of therapies to restore deficient enzyme activity, treatment of MPS focused primarily on prevention and care of complications, still a very important aspect in the management of these patients. In the 80's treatment of MPS with bone marrow transplantation/hematopoietic stem cells transplantation (BMT/HSCT) was proposed and in the 90's, enzyme replacement therapy (ERT),began to be developed and was approved for clinical use in MPS I, II and VI in the first decade of the 21st century. The authors of this paper are convinced that a better future for patients affected by mucopolysaccharidoses depends upon identifying, understanding and appropriately managing the multisystemic manifestations of these diseases. This includes the provision of support measures (which should be part of regular multidisciplinary care of these patients) and of specific therapies. Although inhibition of synthesis of GAG and the recovery of enzyme activity with small molecules also may play a role in the management of MPS, the breakthrough is the currently available intravenous ERT. ERT radically changed the setting for treatment of mucopolysaccharidosis I, II and VI in the last decade., Benefits can even be extended soon to MPS IV A (ERT for this condition is already in clinical development), with prediction for treatment of MPS III A and the cognitive deficit in MPS II by administration of the enzyme directly into the central nervous system (CNS). A large number of Brazilian services, from all regions of the country, already have experience with ERT for MPS I, II and VI. This experience was gained not only by treating patients but also with the participation of some groups in clinical trials involving ERT for these conditions. Summing up the three types of MPS, more than 250 patients have already been treated with ERT in Brazil. The experience of professionals coupled to the data available in international literature, allowed us to elaborate this document, produced with the goal of bringing together and harmonize the information available for the treatment of these severe and progressive diseases, which, fortunately, are now treatable, a situation which bring new perspectives for Brazilian patients, affected by these conditions.Revista da Associação Médica Brasileira 56(3):271-7. · 0.77 Impact Factor -
Article: Pediatric tracheostomy.
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ABSTRACT: To provide an up-to-date review of pediatric tracheostomy, primarily focusing on indications, surgical technique, complications and hospital and home care. MEDLINE and PubMed databases were searched using the following keywords: tracheostomy, tracheotomy, children, newborn. Indications for tracheostomy in children are changing. Today the most common indication is prolonged ventilation. The age at the time of the procedure has also changed, with a peak incidence of tracheostomy in patients less than 1 year old. Except under emergency conditions, pediatric tracheostomy should be performed in the operating room with the child intubated. A horizontal skin incision with vertical tracheal incision and no tracheal resection is recommended. Although post-tracheostomy complications are not uncommon, they usually do not need special treatment or surgical procedures. Tracheostomy mortality can occur in up to 40% of pediatric cases, however the tracheostomy-related mortality rate is only 0 to 6%. The decision to perform a tracheostomy remains complex, and depends on several factors. The procedure is safe and with a low number of complications if carried out at a tertiary hospital by a trained and experienced team.Jornal de pediatria 85(2):97-103. · 1.07 Impact Factor
Top co-authors
Top Journals
Institutions
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2008–2011
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Hospital De Clínicas De Porto Alegre
Porto Alegre, Estado do Rio Grande do Sul, Brazil
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2010
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Great Ormond Street Hospital NHS
London, ENG, United Kingdom -
Faculdade Santo Antônio
Salvador, Estado da Bahia, Brazil
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2002–2010
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Universidade Federal do Rio Grande do Sul
- • Faculdade de Medicina
- • Hospital de Clínicas de Porto Alegre (HCPA)
Porto Alegre, Estado do Rio Grande do Sul, Brazil
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2009
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Hospital Nossa Senhora da Conceição
Tubarão, Estado de Santa Catarina, Brazil -
Universidade de Caxias do Sul (UCS)
Caxias do Sul, Estado do Rio Grande do Sul, Brazil
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