UENIS TANNURI
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Pediatria, Faculdade de Medicina - Docente
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/30 - Laboratório de Investigação em Cirurgia Pediát, Hospital das Clínicas, Faculdade de Medicina - Líder
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/30 - Laboratório de Investigação em Cirurgia Pediát, Hospital das Clínicas, Faculdade de Medicina - Líder
17 resultados
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- Multiple, diffuse schwannomas in a RASopathy phenotype patient with germline KRAS mutation: a causal relationship?(2012) BERTOLA, D. R.; PEREIRA, A. C.; BRASIL, A. S.; SUZUKI, L.; LEITE, C.; FALZONI, R.; TANNURI, U.; POPLAWSKI, A. B.; JANOWSKI, K. M.; KIM, C. A.; MESSIAEN, L. M.
conferenceObject Impact of surgery and chemotherapy over renal function in bilateral Wilms tumor patients(2012) DUARTE, R. J.; CRISTOFANI, L. M.; GIRON, A. M.; DENES, F. T.; ODONE-FILHO, V; TANNURI, U.; SROUGI, M.INTRODUCTION & OBJECTIVES: Bilateral Wilms’ tumor represents 3% of all cases. The challenge of this situation is curing these patients preserving their renal function and quality of life (dialysis free status). Our purpose was to evaluate the success of renal function preservation in children treated for bilateral Wilms’ tumor. MATERIAL & METHODS: From January/1982 through June/2009, 13 children with bilateral Wilms’ tumor (4F: 9M, median age 16 months) were diagnosed. All patients received preoperative chemotherapy with VCR/ACTD (13 patients), VCR/ACTD/ADRIA (10 patients), VCR/ACTD/ADRIA/CBDCA/ETO (3 patients), VCR/ACTD/ADRIA/CBDCA/ETO/IFO (2 patients), for 8 through 40 weeks (median 16 weeks). All patients were submitted to surgical procedure nephron sparing objective. RESULTS: Radical unilatera l nephrectomy was performed in 9 patients (combined with contralateral nodulectomy in 8), bilateral nodulectomy in 3, and unilateral nodulectomy in 1. Two patients presented unilateral disappearing of the disease after preoperative chemotherapy on image and surgical evaluation. The histhopatological analysis revealed the classic triphasic Wilms’ tumor in 9 patients, rhabdomyoblastic differentiation in 3 and blastematous rests in one patient. Four patients relapsed: 2 in the remaining kidney, 1 in the tumoral bed and 1 in lungs (9mo, 1mo, 5mo and 6 mo after surgery). A ll, except the lung relapsed patient, were rescued, achieving a second remission, but one presented a second diffuse abdominal relapse and died. 11/13 patients have normal renal function and are alive and free of disease for 23+ months to 28+years. 1/13 patients presents microalbuminuria and mild systemic hypertension, requiring medication support. CONCLUSIONS: Bilateral Wilms’ tumor was successfully treated and renal function was preserved in 84.6% of surviving patients, even those cases that received nephrotoxic drugs.- Does administering albumin to postoperative gastroschisis patients improve outcome?(2012) TANNURI, Ana Cristina A.; SILVA, Luanna M.; LEAL, Antonio Jose G.; MORAES, Augusto Cesar F. de; TANNURI, UenisOBJECTIVES: Newborns who undergo surgery for gastroschisis correction may present with oliguria, anasarca, prolonged postoperative ileus, and infection. New postoperative therapeutic procedures were tested with the objective of improving postoperative outcome. PATIENTS AND METHODS: One hundred thirty-six newborns participated in one of two phases. Newborns in the first phase received infusions of large volumes of crystalloid solution and integral enteral formula, and newborns in the second phase received crystalloid solutions in smaller volumes, with albumin solution infusion when necessary and the late introduction of a semi-elemental diet. The studied variables were serum sodium and albumin levels, the need for albumin solution expansion, the occurrence of anasarca, the length of time on parenteral nutrition, the length of time before initiating an enteral diet and reaching a full enteral diet, orotracheal intubation time, length of hospitalization, and survival rates. RESULTS: Serum sodium levels were higher in newborns in the second phase. There was a correlation between low serum sodium levels and orotracheal intubation time; additionally, low serum albumin levels correlated with the length of time before the initiation of an oral diet and the time until a full enteral diet was reached. However, the discharge weights of newborns in the second phase were higher than in the first phase. The other studied variables, including survival rates (83.4% and 92.0%, respectively), were similar for both phases. CONCLUSIONS: The administration of an albumin solution to newborns in the early postoperative period following gastroschisis repair increased their low serum sodium levels but did not improve the final outcome. The introduction of a semi-elemental diet promoted an increase in body weight at the time of discharge.
- RET haplotype, not linked to the C620R activating mutation, associated with Hirschsprung disease in a novel MEN2 family(2012) QUEDAS, Elisangela P. S.; LONGUINI, Viviane C.; SEKIYA, Tomoko; COUTINHO, Flavia L.; TOLEDO, Sergio P. A.; TANNURI, Uenis; TOLEDO, Rodrigo A.Hirschsprung disease is a congenital form of aganglionic megacolon that results from cristopathy. Hirschsprung disease usually occurs as a sporadic disease, although it may be associated with several inherited conditions, such as multiple endocrine neoplasia type 2. The rearranged during transfection (RET) proto-oncogene is the major susceptibility gene for Hirschsprung disease, and germline mutations in RET have been reported in up to 50% of the inherited forms of Hirschsprung disease and in 15-20% of sporadic cases of Hirschsprung disease. The prevalence of Hirschsprung disease in multiple endocrine neoplasia type 2 cases was recently determined to be 7.5% and the co-occurrence of Hirschsprung disease and multiple endocrine neoplasia type 2 has been reported in at least 22 families so far. It was initially thought that Hirschsprung disease could be due to disturbances in apoptosis or due to a tendency of the mutated RET receptor to be retained in the Golgi apparatus. Presently, there is strong evidence favoring the hypothesis that specific inactivating haplotypes play a key role in the fetal development of congenital megacolon/Hirschsprung disease. In the present study, we report the genetic findings in a novel family with multiple endocrine neoplasia type 2: a specific RET haplotype was documented in patients with Hirschsprung disease associated with medullary thyroid carcinoma, but it was absent in patients with only medullary thyroid carcinoma. Despite the limited number of cases, the present data favor the hypothesis that specific haplotypes not linked to RET germline mutations are the genetic causes of Hirschsprung disease.
- Transmission of clear cell tumor in a graft liver from cadaveric donor: Case report(2012) BACKES, Ariane N.; TANNURI, Ana Cristina A.; MELLO, Evandro Sobroza de; GIBELLI, Nelson Elias M.; ANDRADE, Wagner de Castro; TANNURI, UenisNeoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donorrecipient serostatus, especially in relation to the EpsteinBarr virus. The patient was a two-yr-old female child with biliary atresia who underwent a liver transplantation from a female cadaver donor. Two adults received kidney transplants from the same donor. Nine months after transplantation, one of the adult recipients developed an urothelial tumor in the kidney graft. Imaging tests were repeated monthly in the liver-transplanted child and revealed no abnormalities. However, one yr and two months after the transplantation, the patient developed episodes of fever. At that time, imaging and liver biopsy showed a clear cell tumor of urothelial origin in the graft and the disease was limited to the liver. The patient underwent liver retransplantation, and she is currently free of tumor recurrence. Although rare, the occurrence of tumors in the post-transplant period from cadaver donors, without previously diagnosed tumors, is one of the many problems encountered in the complex world of organ transplantation.
- Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters(2012) RUANO, R.; TAKASHI, E.; SILVA, M. M. da; CAMPOS, J. A. D. B.; TANNURI, U.; ZUGAIB, M.Objectives To evaluate the accuracy and probabilities of different fetal ultrasound parameters to predict neonatal outcome in isolated congenital diaphragmatic hernia (CDH). Methods Between January 2004 and December 2010, we evaluated prospectively 108 fetuses with isolated CDH (82 left-sided and 26 right-sided). The following parameters were evaluated: gestational age at diagnosis, side of the diaphragmatic defect, presence of polyhydramnios, presence of liver herniated into the fetal thorax (liver-up), lung-to-head ratio (LHR) and observed/expected LHR (o/e-LHR), observed/expected contralateral and total fetal lung volume (o/e-ContFLV and o/e-TotFLV) ratios, ultrasonographic fetal lung volume/fetal weight ratio (US-FLW), observed/expected contralateral and main pulmonary artery diameter (o/e-ContPA and o/eMPA) ratios and the contralateral vascularization index (Cont-VI). The outcomes were neonatal death and severe postnatal pulmonary arterial hypertension (PAH). Results Neonatal mortality was 64.8% (70/108). Severe PAH was diagnosed in 68 (63.0%) cases, of which 63 died neonatally (92.6%) (P < 0.001). Gestational age at diagnosis, side of the defect and polyhydramnios were not associated with poor outcome (P > 0.05). LHR, o/eLHR, liver-up, o/e-ContFLV, o/e-TotFLV, US-FLW, o/eContPA, o/e-MPA and Cont-VI were associated with both neonatal death and severe postnatal PAH (P < 0.001). Receiver-operating characteristics curves indicated that measuring total lung volumes (o/e-TotFLV and US-FLW) was more accurate than was considering only the contralateral lung sizes (LHR, o/e-LHR and o/e-ContFLV; P < 0.05), and Cont-VI was the most accurate ultrasound parameter to predict neonatal death and severe PAH (P < 0.001). Conclusions Evaluating total lung volumes is more accurate than is measuring only the contralateral lung size. Evaluating pulmonary vascularization (Cont-VI) is the most accurate predictor of neonatal outcome. Estimating the probability of survival and severe PAH allows classification of cases according to prognosis.
- Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction in growing animals: immunohistochemical analysis of the expression of TGF-BETA; and VEGF(2012) ANDRADE, Wagner de Castro; SILVA, Luiz Fernando Ferraz da; COELHO, Maria Cecilia de Mendonca; TANNURI, Ana Cristina Aoun; ALVES, Venancio Avancini Ferreira; TANNURI, UenisOBJECTIVE: During the neonatal and infancy periods, some chronic liver diseases may lead to progressive hepatic fibrosis, which is a condition that can ultimately result in the loss of organ function and severe portal hypertension necessitating hepatic transplantation. In a previous report, pharmacological interventions were demonstrated to modulate hepatic fibrosis induced by bile duct ligation in young rats. The administration of pentoxifylline or prednisolone, or the combination of both, resulted in reduced fibrogenesis in portal spaces. The objectives of the present study were to evaluate the expression of transforming growth factor beta and vascular endothelial growth factor after bile duct ligation in young rats and to assess the effect of those same drugs on cytokine expression. METHODS: In this experimental study, 80 young rats (21 or 22 days old) were submitted either to laparotomy and common bile duct ligation or to sham surgery. The animals were allocated into four groups according to surgical procedure, and the following treatments were administered: (1) common bile duct ligation + distilled water, (2) sham surgery + distilled water, (3) common bile duct ligation + pentoxifylline, or (4) common bile duct ligation + prednisolone. After 30 days, a hepatic fragment was collected from each animal for immunohistochemical analysis using monoclonal antibodies against transforming growth factor beta and vascular endothelial growth factor. Digital morphometric and statistical analyses were performed. RESULTS: The administration of pentoxifylline reduced the transforming growth factor beta-marked area and the amount of transforming growth factor beta expressed in liver tissue. This effect was not observed after the administration of prednisolone. There was a significant reduction in vascular endothelial growth factor expression after the administration of either drug compared with the non-treatment group. CONCLUSIONS: The administration of pentoxifylline to cholestatic young rats resulted in the diminished expression of transforming growth factor beta and vascular endothelial growth factor in liver tissue. The administration of steroids resulted in the diminished expression of vascular endothelial growth factor only. These pathways may be involved in hepatic fibrogenesis in young rats submitted to bile duct ligation and exposed to pentoxifylline or prednisolone.
- Effects of selective bile duct ligation on liver parenchyma in young animals: histologic and molecular evaluations(2012) TANNURI, Ana Cristina A.; COELHO, Maria Cecilia M.; GONCALVES, Josiane de Oliveira; SANTOS, Maria Merces; SILVA, Luiz Fernando Ferraz da; BENDIT, Israel; TANNURI, UenisBackground/Purpose: The mechanisms of increased collagen production and liver parenchyma fibrosis are poorly understood. These phenomena are observed mainly in children with biliary obstruction (BO), and in a great number of patients, the evolution to biliary cirrhosis and hepatic failure leads to the need for liver transplantation before adolescence. However, pediatric liver transplantation presents with biliary complications in 20% to 30% of cases in the postoperative period. Intra-or extrahepatic stenosis of bile ducts is frequent and may lead to secondary biliary cirrhosis and the need for retransplantation. It is unknown whether biliary stenosis involving isolated segments or lobes may affect the adjacent nonobstructed lobes by paracrine or endocrine means, leading to fibrosis in this parenchyma. Therefore, the present study aimed to create an experimental model of selective biliary duct ligation in young animals with a subsequent evaluation of the histologic and molecular alterations in liver parenchyma of the obstructed and nonobstructed lobes. Methods: After a pilot study to standardize the surgical procedures, weaning rats underwent ligation of the bile ducts of the median, left lateral, and caudate liver lobes. The bile duct of the right lateral lobe was kept intact. To avoid intrahepatic biliary duct collaterals neoformation, the parenchymal connection between the right lateral and median lobes was clamped. The animals were divided into groups according to the time of death: 1, 2, 3, 4, and 8 weeks after surgical procedure. After death, the median and left lateral lobes (with BO) and the right lateral lobe (without BO [NBO]) were harvested separately. A group of 8 healthy nonoperated on animals served as controls. Liver tissues were subjected to histologic evaluation and quantification of the ductular proliferation and of the portal fibrosis. The expressions of smooth muscle alpha-actin (alpha-SMA), desmin, and transforming growth factor beta 1 genes were studied by molecular analyses (semiquantitative reverse transcriptase-polymerase chain reaction and real-time polymerase chain reaction, a quantitative method). Results: Histologic analyses revealed the occurrence of ductular proliferation and collagen formation in the portal spaces of both BO and NBO lobes. These phenomena were observed later in NBO than BO. Bile duct density significantly increased 1 week after duct ligation; it decreased after 2 and 3 weeks and then increased again after 4 and 8 weeks in both BO and NBO lobes. The portal space collagen area increased after 2 weeks in both BO and NBO lobes. After 3 weeks, collagen deposition in BO was even higher, and in NBO, the collagen area started decreasing after 2 weeks. Molecular analyses revealed increased expression of the alpha-SMA gene in both BO and NBO lobes. The semiquantitative and quantitative methods showed concordant results. Conclusions: The ligation of a duct responsible for biliary drainage of the liver lobe promoted alterations in the parenchyma and in the adjacent nonobstructed parenchyma by paracrine and/or endocrine means. This was supported by histologic findings and increased expression of alpha-SMA, a protein related to hepatic fibrogenesis.
conferenceObject Histopathological markers as clinical indicators of early liver transplantation in biliary atresia(2012) LONGO-SANTOS, L. R.; TEODORO, W.; VELOSA, A. P.; MELLO, E.; PARRA, E.; COELHO, M. C.; ALVES, V.; CAPELOZZI, V.; TANNURI, U.Objective: Biliary atresia (BA) is the most common neonatal cholestatic disorder and the prime indication for liver transplantation (LTx) in children. Histopathological markers in liver biopsies emerge promise as indicator of early LTx in patients with BA. Method: Ductular proliferation, ductal plate malformations and type I, III, IVand V collagen deposition were evaluated on Kasai portoenterostomy (KPE) and liver transplantation (LTx) biopsies from 36 children with BA. Formalin fixed and paraffin embedded liver biopsies were stained with hematoxylin-eosin, picrosirius-polarization and immunofluorescence methods. There were analyzed liver histoarchitecture, biliary ductus and collagen deposition in hepatic compartments. Pathologic findings were graded according to a 5-point semi-quantitative severity-based scoring system. Impact of these markers was tested on LTx time (<2 year and >2 year). Results: Median age of KPE was 12 weeks (range 6–20) and of LTx was 27 months (range 6–120). In KPE liver biopsies, ductular proliferation, ductal plate malformations and collagen deposition were increased but these parameters presented no association with clinical evolution for early LTx. Furthermore, collagen V prominent deposition was found along of hepatic sinusoids and type I, III and IV were more frequent in portal compartment. Conclusion: These results suggest that histopathological parameters evaluation presented may not determine early LTx in biliary atresia.- Nutritional risk and anthropometric evaluation in pediatric liver transplantation(2012) ZAMBERLAN, Patricia; LEONE, Claudio; TANNURI, Uenis; CARVALHO, Werther Brunow de; DELGADO, Artur FigueiredoOBJECTIVE: To analyze the nutritional status of pediatric patients after orthotopic liver transplantation and the relationship with short-term clinical outcome. METHOD: Anthropometric evaluations of 60 children and adolescents after orthotopic liver transplantation, during the first 24 hours in a tertiary pediatric intensive care unit. Nutritional status was determined from the Z score for the following indices: weight/age, height/age or length/age, weight/height or weight/length, body mass index/age, arm circumference/age and triceps skinfold/age. The severity of liver disease was evaluated using one of the two models which was adequated to the patients' age: 1. Pediatric End-stage Liver Disease, 2. Model for End-Stage Liver Disease. RESULTS: We found 50.0% undernutrition by height/age; 27.3% by weight/age; 11.1% by weight/height or weight/length; 10.0% by body mass index/age; 61.6% by arm circumference/age and 51.0% by triceps skinfold/age. There was no correlation between nutritional status and Pediatric End-stage Liver Disease or mortality. We found a negative correlation between arm circumference/age and length of hospitalization. CONCLUSION: Children with chronic liver diseases experience a significant degree of undernutrition, which makes nutritional support an important aspect of therapy. Despite the difficulties in assessment, anthropometric evaluation of the upper limbs is useful to evaluate nutritional status of children before or after liver transplantation.