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1.
Pediatr Hematol Oncol ; 27(3): 161-78, 2010 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-20367260

RESUMO

AIM: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. METHODS AND PATIENTS: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III-IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II-IV UH tumors, VCR + Act-D + ADR + etoposide + RT. RESULTS: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases < or =2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. CONCLUSIONS: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.


Assuntos
Neoplasias Renais/terapia , Tumor de Wilms/terapia , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Lactente , Recém-Nascido , Neoplasias Renais/mortalidade , Masculino , Tumor de Wilms/mortalidade
2.
Pediatr Blood Cancer ; 54(7): 885-9, 2010 Jul 01.
Artigo em Inglês | MEDLINE | ID: mdl-20049935

RESUMO

AIM: Thoracic neuroblastic tumors (NBTs) are reported to have better prognosis. We aimed to review clinical characteristics, treatment results, and outcome of our patients with thoracic NBT. PATIENTS AND METHODS: Files of 87 children treated at our hospital between 1973 and 2007 with the diagnoses of thoracic NBT were reviewed for clinical and pathological characteristics. Treatment results and outcomes of these cases were examined. RESULTS: All but one tumors were located in posterior mediastinum, one in the posterior chest wall. Median age of all was 2.1 years (range, 0.03-14; F/M: 1.42). Fifteen cases had ganglioneuromas (GN), 26 ganglioneuroblastomas (GNBL), and 46 neuroblastomas (NBLs). Stages were: I, 20.5%; II, 22.1%; III, 38.2%; IV, 14.7%; IVS, 4.5%. Stages III and IV were more common in cases over 1 year of age. In 20 patients diagnoses were incidental. Twenty-two of 87 (25.3%) had symptomatic spinal cord compression and 15 (17.3%) had Horner syndrome. Ten-year overall and event-free survival rates were 71.2% and 67.4%, respectively. Survival rates did not differ depending on the age being younger or older than 1 year. Ten-year survival rates were 88.8% in stages I, II, IVS; 65.3% in stage III and 27.8% in stage IV (P = 0.0002). CONCLUSIONS: Thoracic NBLs had a favorable prognosis. This might be a result of earlier diagnosis and some distinct biological characteristics. Favorable prognosis would suggest less aggressive treatment for such patients. Further studies on the biological characteristics of NBLs in the thoracic site and their association with outcome should be done.


Assuntos
Neuroblastoma/patologia , Neoplasias Torácicas/patologia , Adolescente , Antineoplásicos/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Estadiamento de Neoplasias , Neuroblastoma/mortalidade , Neuroblastoma/terapia , Prognóstico , Radioterapia , Estudos Retrospectivos , Neoplasias Torácicas/mortalidade , Neoplasias Torácicas/terapia , Procedimentos Cirúrgicos Torácicos , Resultado do Tratamento
3.
J Pediatr Hematol Oncol ; 31(7): 471-80, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19564739

RESUMO

OBJECTIVE AND METHOD: The survival of the patients with neuroblastoma has improved in last few decades. But still it depends on various clinical and biological factors. To assess the clinical features and trends in survival, the data for 500 newly diagnosed patients between January 1972 and December 2004 from a single center were retrospectively analyzed. RESULTS: Histopathologic subtypes were neuroblastoma (NBL) in 462 patients (92.4%) and ganglioneuroblastoma in 38 patients (7.6%). The median age was 2.9 years and Male/Female ratio was 1.3/1. Primary tumor sites were abdomen, thorax, pelvis, neck, and others with the frequency of 72.2%, 14.9%, 3.8%, 3.2%, and 5.9%, respectively. There were 30, 49, 133, 257, 31 patients with stage 1, 2, 3, 4, 4S disease and their 10-year survival rates were 100%, 75.8%, 34.1%, 6.5%, and 59.4%, respectively. The outcome has significantly improved according to 10-year periods. The 5-year overall survival rates were 14%, 26.1%, 39.2%, and 52.4% for the years of 1970s, 1980s, 1990s, and after 2000. Surgical procedure involving total or near total tumor removal improved the survival (P=0.002). Both 5-year overall survival and event free survival rates were higher when partial resection was performed, especially in stage 3 disease (P=0.002 and P=0.02). In multivariate analysis, age above 18 months at diagnosis (P=0.01), stage 4 disease (P<0.001), abdominal primary tumor site (P<0.001), NBL subtype in histopathology (P=0.001), responsiveness to chemotherapy (P<0.001) positive or high Vanillyl mandelic acid levels (P=0.02) and male sex (P=0.008) were the determinants of poor prognosis. CONCLUSIONS: The survival rates in children with local disease are comparable with the results of developed countries; however, the results in children with advanced disease are still not satisfactory. To improve the outcome, especially in children with advanced disease, more effective chemotherapy regimens and molecular therapies should be investigated. Sharing the knowledge and capacity building to improve the treatment results in NBL are also critical for developing countries.


Assuntos
Neuroblastoma/mortalidade , Neuroblastoma/patologia , Adolescente , Distribuição por Idade , Idade de Início , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Terapia Combinada , Países em Desenvolvimento/estatística & dados numéricos , Feminino , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Estadiamento de Neoplasias , Neuroblastoma/terapia , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Fatores Sexuais , Taxa de Sobrevida , Turquia
4.
Turk J Pediatr ; 50(4): 336-41, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-19014046

RESUMO

In this research, we aimed to evaluate the accordance of radiologic study results with each other and with surgical findings with regard to presence and/or absence of intussusception. One hundred and seventy-nine patients treated for intussusception between 1993 and 2003 inclusive were retrospectively reviewed to compare results of initial ultrasonography, colonography, followup ultrasonography after conservative management (reduction with barium enema and/or air insufflation) and surgical findings to determine their accordance within each other with regard to diagnosis of intussusception. Results of initial ultrasonography were not in accordance with results of colonography. There was accordance between conservative management and follow-up ultrasonography results. Conservative management results were not in accordance with surgical findings. There was no accordance between followup ultrasonography results and surgical findings. Discordance of radiologic examination results with each other and with surgical findings indicates that intussusception is still a clinical diagnosis and clinical parameters deserve more importance in surgical decision-making. Radiologic examinations should be considered as complementary studies, not as definitive discriminators of childhood intussusception to achieve appropriate diagnosis and treatment.


Assuntos
Intussuscepção/diagnóstico por imagem , Intussuscepção/cirurgia , Pré-Escolar , Colonoscopia , Feminino , Humanos , Lactente , Intussuscepção/terapia , Masculino , Radiografia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Ultrassonografia
5.
Surg Today ; 38(8): 726-30, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18668316

RESUMO

Progressive familial intrahepatic cholestasis (PFIC) is a cholestatic liver disease of childhood. Pruritus secondary to increased bile salts in the serum may not respond to medical treatment. Partial external biliary diversion (PEBD), which reduces the serum bile salt level in the enterohepatic cycle, is used in the treatment of this symptom. In this study, our experience in performing this technique and the early promising results of PEBD in two children with PFIC are reported along with a review of the current literature. Partial external biliary diversion was performed by interposing a 15-cm jejunum between the gallbladder and abdominal wall. Biliary drainage through a stoma began in the fi rst postoperative day and reached 120-200 ml/day. Pruritus improved and then stopped on the 15th postoperative day, while the serum bile acid concentration also decreased. Partial external biliary diversion by jejunal interposition provides an excellent control of pruritus in children with PFIC with no adverse effects. A cholecystectomy should therefore be avoided in patients with PFIC.


Assuntos
Procedimentos Cirúrgicos do Sistema Biliar/métodos , Colestase Intra-Hepática/cirurgia , Prurido/cirurgia , Criança , Pré-Escolar , Colestase Intra-Hepática/complicações , Colestase Intra-Hepática/genética , Feminino , Humanos , Masculino , Prurido/etiologia
6.
Urol Int ; 81(1): 41-6, 2008.
Artigo em Inglês | MEDLINE | ID: mdl-18645270

RESUMO

AIM: To clarify the factors affecting the success rate of endoscopic subureteral injection (ESI) treatment of vesicoureteral reflux (VUR). METHOD: All patients who had ESI treatment for VUR at our unit between 1999 and 2002 were retrospectively reviewed with regard to age, gender, causes of VUR, reflux grade, type of injected materials, number of injections, results of cystourethrograms (VCUG), and outcome. The injected implants were gluteraldehyde cross-linked bovine collagen (GclBC), dextranomers in sodium hyaluronan (DiSH) and calcium hydroxylapatite (CH). A successful result was defined as absence of VUR confirmed by VCUG performed 3 months after the ESI procedure. Basic descriptive statistics were performed along with the chi(2) test (p < 0.05 was significant). RESULTS: 50 children (81 ureters) consisting of 27 girls (43 ureters) and 23 boys (38 ureters) with a mean age of 7.4 +/- 4.6 years constituted the study group. Grade III VUR was the most prominent grade (42%, 34 ureters) noted in all age groups. There was no significant difference between boys and girls with regard to the number of each grade of VUR. The success rate of first ESI procedure was 55.6% and was similar in each grade of VUR varying from 50 to 66%. Repeated injections have resulted in an overall success rate of 84%. No significant difference was noted between the age groups with regard to the success rate of ESI. Repeated ESI procedures were found to be unsuccessful in grade V VUR when compared to other grades (p < 0.05).There was a significant difference between primary reflux (76.5%, 62 ureters) and exstrophic (21%, 17 ureters) patients with regard to the grade of VUR and success rate of ESI. Exstrophic patients presented with a higher incidence of grade V VUR (41%) and with a lower success rate of ESI (64.7%). DiSH was the most commonly used agent (47 ureters) followed by CH (22 ureters) and GclBC (12 ureters). The success rates of the ESI procedure by each material were 91.5, 81.8, and 58.3%, respectively. No significant difference was noted between DiSH and CH. Low success rates by GclBCwere attributed to less usage of the material. 82% of the ESI procedures were done by surgeon A (49 ureters) and B (18 ureters) with a success rate of 96 and 72%, respectively. No significant difference was noted between A and B with regard to the success rate and type of injected material and the success rate in each grade of VUR (p > 0.05). No untoward effects were noted in short- and long-term follow-up (mean 4 +/- 1.2 years) of any patient. CONCLUSION: The ESI procedure provides a high success rate for the treatment of VUR which decreases in grade V VUR and presence of exstrophia vesica. Single injection of various materials has been found to be successful in most of the patients with grade II VUR, whereas grade IV and III patients have required repeated injections which have resulted in 100 and 94% success rates, respectively. Grade I VUR can be managed by close follow-up and appropriate antibiotic therapy without any surgical and/or endoscopic intervention. Patients presenting with grade V VUR should undergo open surgery if the first trial of ESI procedure results in failure as repeated injections have proved to be unsuccessful in this grade. The success rate of the ESI procedure does not seem to be affected by the type of injected material and different surgeons performing the procedure after achieving the learning curve.


Assuntos
Endoscopia/métodos , Refluxo Vesicoureteral/cirurgia , Refluxo Vesicoureteral/terapia , Adolescente , Animais , Cálcio/química , Bovinos , Criança , Pré-Escolar , Colágeno/química , Colágeno/metabolismo , Reagentes de Ligações Cruzadas/farmacologia , Durapatita/química , Glutaral/farmacologia , Humanos , Ácido Hialurônico/química , Resultado do Tratamento
7.
Turk J Pediatr ; 49(2): 226-30, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17907529

RESUMO

We aimed in this study to evaluate the clinical and radiological features of the late recurrence of Wilms' tumor in children. Among 553 children diagnosed with Wilms' tumor between 1972 and 2004, four cases were determined to be late recurrences. Clinical, histopathological parameters, treatment details, and outcomes of the patients were evaluated retrospectively. The ages of the patients at the time of diagnosis were 2, 5, 5, and 9 years and the male/female ratio was 1/3. Two patients had stage II disease and two had stage IV characteristics. Histopathological examination showed favorable histology in all of the patients. Initial treatment was surgery and chemotherapy, which included vincristine and actinomycin-D. Abdominal radiotherapy was performed in two patients. Recurrence times were 36, 41, 51, and 96 months. Local recurrence and lung metastasis were detected in two patients, local recurrence in one, and lung nodules in the fourth patient. At the time of relapse, the chemotherapy protocols were as follows: vincristine, actinomycin-D, adriamycin, and cyclophosphamide in two patients; vincristine, actinomycin-D, and epirubicin in one patient; and vincristine, actinomycin-D, and adriamycin in the last patient. In the cases with late local recurrence, one patient had a local spillage and one patient had regional lymph node involvement. Although the other patient had local spillage, regional lymph node involvement, and renal artery invasion, isolated lung recurrence was observed. Only one patient had progressive disease and is still under treatment, whereas the other patients died with disease. Major recurrence sites were both local and the lungs. All of the patients had regional features including spillage, regional lymph node involvement, and vascular or capsular involvement. Late recurrence in patients with Wilms' tumor is a poor prognostic factor and should be treated with an intensified regimen.


Assuntos
Neoplasias Renais/patologia , Tumor de Wilms/patologia , Criança , Pré-Escolar , Evolução Fatal , Feminino , Humanos , Lactente , Neoplasias Pulmonares/secundário , Metástase Linfática , Masculino , Recidiva Local de Neoplasia , Estadiamento de Neoplasias , Prognóstico
8.
Pediatr Surg Int ; 23(8): 755-61, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17569061

RESUMO

Ingestion of a foreign body (FB) is a prevalent condition among children. The type of FB varies according to the feeding habits and sociocultural features of communities. The management modality differs also between disciplines due to use of conventional techniques. We aimed to picture the general characteristics of FB ingestion and treatment alternatives, to mention the indications of open surgery in an advanced pediatric surgical center. The records of patients who were hospitalized for FB ingestion between 1973 and May 2005 were evaluated retrospectively. One hundred and twelve patients were enrolled into the study. The mean age was 2.27 +/- 2.84 years with a M/F ratio of 59/53. The history was suggestive of ingestion in 92% of patients. The age did not differ significantly whether the history was positive or negative (3.6 years vs. 4.8 years and P = 0.19). Most common presenting symptom was vomiting (28.6%). The duration of symptoms was longer in patients with negative history (median 47.7 h vs. 28.1 h and P < 0.002). Physical examination was normal in 89.3% of cases. Most common localization of the FB shown in plain X-ray was the esophagus (67%). Esophagography revealed nonopaque FB in the esophagus in 4.4%. X-ray was normal in 6.3% of the patients. The age of patient did not determine the localization of FB on admission (P = 0.436). Endoscopic removal was attempted in 75% and was successful in 68% of patients in which FB was extracted by using laryngoscope and Magill forceps (12%), rigid esophagoscope with FB forceps (51%), and flexible endoscope with FB forceps (5%). FB could not be found in 32% of patients at initial rigid esophagoscopy or flexible endoscopy. FB was eliminated spontaneously (n = 19) or extracted surgically (n = 8). Follow-up was preferred in 21% of patients on initial admission. FB proceeded uneventfully in 15 patients or was extracted by flexible endoscopy or surgery in one and eight patients, respectively. Surgery was performed in 4% on admission. Surgery or endoscopy were essentially required in cases whose follow-up period exceeded 4 days when compared with patients who eliminated FB spontaneously within 4 days, independent to the location of FB. The metallic objects were the frequently ingested FBs (83.8%) in which the safety pins (SPs) (n = 53) and coins (n = 25) were the most frequent. The type of FB did not affect the FB localization on admission (P = 0.38). The duration of hospitalization was longer in patients with delayed admission; 2.46 +/- 3.51, 3.80 +/- 8.17, and 5.72 +/- 4.24 days for the admissions within first, second-fifth days, and sixth or later days after ingestion, respectively (P = 0.000). Pediatric surgery has the largest spectrum of duty in the treatment of FB ingestion in children. Negative history, normal physical examination findings and absence of symptoms do not exclude the possibility of FB ingestion. Presentation with isolated respiratory symptoms is an enigma that can lead to misdiagnoses. The mode of management should be selected according to the patient's condition, surgeon's experience, and available technical equipment as well as the location and type of ingested FB. Especially, SPs should be treated by experienced surgeons. Simple extraction techniques and both rigid and flexible endoscopies with appropriate forceps as well as surgery can be used for the extraction of FBs lodged in the alimentary tract. Surgery can be expected especially in asymptomatic cases that have been followed up for more than 4 days irrespective of the location of FB.


Assuntos
Sistema Digestório , Corpos Estranhos/complicações , Corpos Estranhos/terapia , Adolescente , Algoritmos , Criança , Pré-Escolar , Endoscopia do Sistema Digestório , Feminino , Corpos Estranhos/cirurgia , Humanos , Lactente , Masculino , Estudos Retrospectivos
10.
Pediatr Surg Int ; 23(8): 811-4, 2007 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-17347840

RESUMO

A newborn case with cardiac rhabdomyoma is presented to discuss the differential diagnosis of mediastinal tumors around the heart in children. The diagnosis of rhabdomyoma can be suggested by modern visualization methods such as computerized tomography, magnetic resonance imaging and echocardiography. However, surgery is clearly indicated in cases with obstruction of flow tracts, valvular dysfunction and dysrhythmia, and in cases with indefinite diagnosis, or in the absence of association with tuberous sclerosis. Cardiac rhabdomyoma should be included in the differential diagnosis of masses located adjacent to the heart in infants.


Assuntos
Neoplasias Cardíacas/diagnóstico , Neoplasias do Mediastino/diagnóstico , Rabdomioma/diagnóstico , Diagnóstico Diferencial , Humanos , Recém-Nascido , Masculino
11.
Nephron Clin Pract ; 105(2): c58-61, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17135769

RESUMO

OBJECTIVE: To evaluate the clinical features and outcome of children with renal cell carcinoma (RCC). PATIENTS AND METHODS: Eleven patients with RCC who were diagnosed between 1972 and 2004 were retrospectively analyzed. Clinical features, histopathology, treatment regimens and outcomes of the patients were evaluated. RESULTS: The male/female ratio was 3:8, with a median age of 10 years. The stage distribution was as follows: 3 patients in stage I, 1 patient in stage II, 3 patients in stage IIIb, and 4 patients in stage IV. Five of 7 patients with stage II-IV received an actinomycin D-based regimen, one received a cisplatin-based regimen, and the other was given 5-fluorouracil (5-FU). In the last patient, interferon-alpha was given in combination with 5-FU; 1 of the patients on the actinomycin D regimen received interferon-alpha as well. All of the stage I patients are alive without disease. Three patients with stage IIIb, stage IV and stage II disease are alive without disease 8, 14 and 26 years after their diagnosis, respectively. The other stage IV and stage IIIb patients died of the disease. CONCLUSION: Nephroureterectomy is the main treatment modality, and it is sufficient for stage I patients. For patients with stage II-IV RCC, interferon-alpha and/or actinomycin D-based chemotherapy is the treatment of choice.


Assuntos
Carcinoma de Células Renais/patologia , Carcinoma de Células Renais/terapia , Neoplasias Renais/patologia , Neoplasias Renais/terapia , Adolescente , Antibióticos Antineoplásicos/uso terapêutico , Antimetabólitos Antineoplásicos/uso terapêutico , Antineoplásicos/uso terapêutico , Carcinoma de Células Renais/mortalidade , Carcinoma de Células Renais/secundário , Criança , Pré-Escolar , Cisplatino/uso terapêutico , Dactinomicina/uso terapêutico , Feminino , Fluoruracila/uso terapêutico , Seguimentos , Humanos , Neoplasias Renais/mortalidade , Masculino , Estadiamento de Neoplasias , Nefrectomia , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento , Ureter/cirurgia
12.
Pediatr Surg Int ; 23(2): 183-7, 2007 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-17043874

RESUMO

To investigate the short and long term histopathological alterations caused by submucosal injection of dextranomers in sodium hyaluronan (DiHA) based on an experimental rat model. Sixty Sprague-Dawley rats were assigned into two groups as group I and II, each containing 30 rats. Totally 0.1 ml of saline solution and 0.1 ml of DiHA were injected into the submucosa of bladder of first (control) and second groups, respectively. Both group I and II were further subdivided into three other groups as Group IA, IB, IC and Group IIA, IIB, IIC according to the sacrificial period. Group IA and IIA, IB and IIB, IC and IIC rats (ten rats for each group) were sacrificed 3, 6, and 12 months after surgical procedure, respectively. Two slides prepared from injection site of the bladder were evaluated completely for each rat by being unaware of the groups and at random by two independent senior pathologists to determine the fibroblast invasion, collagen formation, capillary growth and inflammatory reaction. Additionally, randomized brain sections from each rat were also examined to detect migration of the injection material. The measurements were made using an ocular micrometer at 10x magnification. The results were assessed using t-tests for paired and independent samples, with P<0.05 considered to indicate significant differences; all values were presented as the mean (SD). Migration to the brain was not detected in any group. Significant histopathological changes in the DiHA injected groups were granuloma formation in 43.3%, foreign body type giant cells in 76.6%, inflammatory infiltration in 100%, and fibroblasts surrounding microspheres in 100% of the rat bladder. The interaction between granuloma formation and long-term tissue effects in children is still obscure. We emphasize that further prospective human (and/or animal) studies are required to clarify the long-term effects of granuloma formation with regard to clinical applications.


Assuntos
Anticoagulantes/farmacologia , Dextranos/farmacologia , Granuloma/etiologia , Ácido Hialurônico/farmacologia , Refluxo Vesicoureteral/tratamento farmacológico , Animais , Anticoagulantes/administração & dosagem , Encéfalo/patologia , Dextranos/administração & dosagem , Granuloma/patologia , Ácido Hialurônico/administração & dosagem , Injeções , Masculino , Ratos , Ratos Sprague-Dawley , Bexiga Urinária/patologia , Refluxo Vesicoureteral/patologia
13.
Respiration ; 74(3): 335-7, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-15988170

RESUMO

Pulmonary lymphangioleiomyomatosis (PLAM) is a rare, diffuse progressive interstitial lung disease that affects females of childbearing age and is characterized by diffuse proliferation of abnormal smooth muscle fibers predominantly developing in the lung and leading to cystic destruction. A prepubertal 13-year-old girl with PLAM associated with renal and hepatic angiomyolipomas who was treated by nephroureterectomy and thoracoscopic pleurodesis is presented. To the best of our knowledge, this is the first reported case of PLAM associated with renal and hepatic angiomyolipomas at the prepubertal age. After evaluating the clinicopathologic features of this rare entity, the authors conclude that PLAM should be considered in the differential diagnosis of cystic pulmonary pathologies in children, particularly teenagers. The most important clinical aid is to bear this rare entity in mind when a child presents with renal and/or hepatic angiomyolipomas. Thoracoscopic pleurodesis is the most effective treatment modality for recurrent pneumothoraces.


Assuntos
Angiomiolipoma , Neoplasias Renais , Neoplasias Hepáticas , Neoplasias Pulmonares , Linfangioleiomiomatose , Neoplasias Primárias Múltiplas , Adolescente , Angiomiolipoma/diagnóstico por imagem , Angiomiolipoma/patologia , Feminino , Seguimentos , Humanos , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/patologia , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/patologia , Neoplasias Pulmonares/diagnóstico por imagem , Neoplasias Pulmonares/patologia , Linfangioleiomiomatose/diagnóstico por imagem , Linfangioleiomiomatose/patologia , Neoplasias Primárias Múltiplas/diagnóstico por imagem , Neoplasias Primárias Múltiplas/patologia , Tomografia Computadorizada por Raios X
14.
J Pediatr Surg ; 41(11): 1899-902, 2006 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-17101367

RESUMO

BACKGROUND/PURPOSE: A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease. METHODS: Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome. RESULTS: Patients with PVT (n = 4, 5.88%) and without PVT (n = 64, 94.2%) had a mean age and female/male ratio of 13.2 years (range, 10-16 years) and 4:0, and 10.2 years (range, 1-16 years) and 29:35, respectively. Postoperative thrombocyte levels and splenic mass with and without PVT was 804 x 10(3)/mm(3) and 752.5 g, and 465.2 x 10(3)/mm(3) and 441g, respectively. Three patients with PVT presented with abdominal pain, fever, and vomiting. The diagnosis of PVT was made by Doppler ultrasonography in all patients including the asymptomatic case. Protein C, protein S, and antithrombin III levels were mostly decreased and/or normal and di-dimer levels were increased and/or normal after the development of PVT. Antiplatelet (acetylsalicylic acid) and antithrombotic therapy (low molecular weight heparin) were treatment agents. None of the patients needed surgery. During a mean follow-up period of 55.5 months, by Doppler ultrasonography, 1 patient was found to be free of thrombosis, whereas 1 had partial thrombosis. Two patients developed cavernomatous transformation leading to portal hypertension. CONCLUSIONS: Portal vein thrombosis is a rare but significant complication of splenectomy done for hematologic diseases. According to our results, female gender and decreased levels of coagulation inhibitors seem to be risk factors in addition to previously mentioned thrombocytosis and greater splenic mass. Doppler ultrasonography may be performed in all patients after splenectomy to screen PVT. In the presence of well-known risk factors, prophylactic antiplatelet and antithrombotic therapy should be considered after splenectomy.


Assuntos
Doenças Hematológicas/cirurgia , Veia Porta , Esplenectomia/efeitos adversos , Trombose Venosa/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
15.
J Pediatr Hematol Oncol ; 28(7): 423-6, 2006 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-16825987

RESUMO

To analyze changes in the overall survival (OS) rate of children with Wilms tumor treated in a single institute over nearly 30 years. This study included 327 children with a newly diagnosed Wilms tumor. Their median age was 3 years, and the male:female ratio was 1.1. Survival rates were analyzed according to the stage of disease, histopathology, and different treatment regimens used between 1972 and 1999. At diagnosis, 51.1% of patients had advanced stage disease. Ten patients had anaplasia, and; 97% (317 patients) of the tumors had favorable histopathology. The 10-year OS rate was 60.6% for the entire group, but varied according to the years in which the patients were treated, the chemotherapy regimen, and stage of disease. Patients treated during the periods of 1972 to 1979, 1980 to 1989, and 1990 to 1999 had 10-year OS rates of 48.5%, 64.3%, and 72.8%, respectively. The 10-year OS rate in children treated with actinomycin only was 36.7% compared with 48% for children treated with the actinomycin-D+vincristine regimen with a 3-month interval, 67% for the actinomycin-D+vincristine regimen with a 1.5-month interval, 54.5% for the poor-risk regimen (actinomycin-D, vincristine, cyclophosphamide, and adriamycin), and 53.4% for the SIOP-9 protocol. Children with stage I to IV disease had 10-year OS rates of 75%, 77.1%, 54.4%, and 30.4%, respectively. The 10-year OS rates for children with stage III and IV disease increased from 46.4% and 13.4% for patients treated between 1972 to 1979 period to 75% and 54.5% for children treated during 1990 to 1999. The 10-year OS rate for children with Wilms tumor improved as treatment strategies evolved, illustrating that pediatric oncology in Turkey is developing parallel to the Western world.


Assuntos
Neoplasias Renais/patologia , Tumor de Wilms/patologia , Adolescente , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Neoplasias Renais/diagnóstico , Neoplasias Renais/terapia , Masculino , Estadiamento de Neoplasias , Prognóstico , Taxa de Sobrevida , Tempo , Resultado do Tratamento , Turquia , Tumor de Wilms/diagnóstico , Tumor de Wilms/terapia
16.
Pediatr Transplant ; 10(4): 517-20, 2006 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-16712614

RESUMO

To evaluate clinical and laboratory findings of these patients and the efficacy of liver transplantation in children with hepatocellular carcinoma (HCC) and hepatoblastoma (HB) associated with tyrosinemia. Among 113 children with liver tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular clinic visits for tyrosinemia, elevated alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given chemotherapy including cisplatin and adriamycin. In three patients, living-related liver transplantation was performed. They had no treatment after transplantation. All of them are disease free. One patient was treated with chemotherapy and right hepatectomy. She had no suitable donor for living-related liver transplantation. Three months after completing chemotherapy, she had recurrent tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for tyrosinemia. In these patients liver transplantation is curative both for liver tumor and tyrosinemia.


Assuntos
Carcinoma Hepatocelular/enzimologia , Carcinoma Hepatocelular/cirurgia , Neoplasias Hepáticas/enzimologia , Neoplasias Hepáticas/cirurgia , Transplante de Fígado , Tirosinemias/cirurgia , Adolescente , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/diagnóstico por imagem , Carcinoma Hepatocelular/patologia , Criança , Intervalo Livre de Doença , Evolução Fatal , Feminino , Seguimentos , Humanos , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/diagnóstico por imagem , Neoplasias Hepáticas/patologia , Masculino , Radiografia , Recidiva , Fatores de Tempo , Resultado do Tratamento , Tirosinemias/complicações , Ultrassonografia
17.
BMC Urol ; 6: 3, 2006 Feb 18.
Artigo em Inglês | MEDLINE | ID: mdl-16503996

RESUMO

BACKGROUND: The aim of present study is to investigate the short and long term histopathological alterations caused by submucosal injection of gluteraldehyde cross-linked bovine collagen based on an experimental rat model. METHODS: Sixty Sprague-Dawley rats were assigned into two groups as group I and II each containing 30 rats. 0.1 ml of saline solution and 0.1 ml of gluteraldehyde cross-linked bovine collagen were injected into the submucosa of bladder of first (control) and second groups, respectively. Both group I and II were further subdivided into 3 other groups as Group IA, IB, IC and Group IIA, IIB, IIC according to the sacrification period. Group IA and IIA, IB and IIB, IC and IIC rats (10 rats for each group) were sacrificed 3, 6, and 12 months after surgical procedure, respectively. Two slides prepared from injection site of the bladder were evaluated completely for each rat by being unaware of the groups and at random by two independent senior pathologists to determine the fibroblast invasion, collagen formation, capillary ingrowth and inflammatory reaction. Additionally, randomized brain sections from each rat were also examined to detect migration of the injection material. The measurements were made using an ocular micrometer at x10 magnification. The results were assessed using t-tests for paired and independent samples, with p < 0.05 considered to indicate significant differences; all values were presented as the mean (SD). RESULTS: Migration to the brain was not detected in any group. Significant histopathological changes in the gluteraldehyde cross-linked bovine collagen injected groups were fibroblast invasion in 93.3%, collagen formation in 73.3%, capillary ingrowth in 46.6%, inflamatory reaction in 20%. CONCLUSION: We emphasize that the usage of gluteraldehyde cross-linked bovine collagen in children appears to be safe for endoscopic treatment of vesicoureteral reflux.


Assuntos
Encéfalo/patologia , Colágeno/farmacologia , Reagentes de Ligações Cruzadas/farmacologia , Bexiga Urinária/patologia , Animais , Materiais Biocompatíveis/farmacologia , Encéfalo/efeitos dos fármacos , Fibroblastos/patologia , Reação a Corpo Estranho/etiologia , Reação a Corpo Estranho/patologia , Inflamação/etiologia , Inflamação/patologia , Injeções , Masculino , Modelos Animais , Ratos , Ratos Sprague-Dawley , Bexiga Urinária/efeitos dos fármacos
18.
Pediatr Surg Int ; 22(3): 228-32, 2006 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-16395609

RESUMO

Hepatic resection is the main treatment modality for hepatic tumors in childhood. Advances in diagnostic technique, preoperative preparation, surgical technique, and postoperative management increased the success rate. The aim of this study is to report our experience in hepatic lobectomy, which is relatively rare procedure in childhood. Medical records of 25 patients who underwent hepatic lobectomy between January 1977 and June 2002 were reviewed retrospectively. Age, gender, diagnosis, physical examination findings, results of preoperative laboratory investigations, radiological examination, resectability criteria, preoperative biopsies, chemotherapies, radiotherapies, postoperative pathological results, incisions, operation technique, intraoperative transfusions, drains used, antibiotic prophylaxes, and intraoperative and postoperative complications were evaluated for all patients. Out of 25 patients with hepatic tumor seven patients with hepatoblastoma and four patients with hepatocellular carcinoma were given 5.7 +/- 0.3 cycles of chemotherapy before the operation. Right lobectomy (n = 12), left lobectomy (n = 5), extended left lobectomy (n = 4), and extended right lobectomy (n = 3) and right lobectomy with enucleation of two masses from left lobe (n = 1) were performed. Intraoperative blood transfusion of 30.7+/-6.0 ml/kg body weight was necessary. Pathological examination of resected tumors revealed hepatoblastoma (n=11), mesenchymal hamartoma (n = 5), hepatocellular carcinoma (n = 4), hemangioendothelioma (n=1), malignant mesenchymal tumor (n = 1), hemangioma (n = 1), cyst adenoma (n = 1), and metastasis of cellular mesoblastic nephroma (n = 1). Patients were observed in the intensive care unit for 3.4 +/- 0.3 days. Postoperative complications were sepsis (n = 1), disseminated intravascular coagulation (n = 2), fever (n = 3), jaundice (n = 3), intraabdominal abscess (n = 3), ileus (n = 2), and subdiaphragmatic abscess with pleural effusion (n = 1). Hepatic lobectomy is a major operation, which is feasible yielding curative results in children. Safe hepatic resections with acceptable blood loss can be performed by a technique relying on good anatomic dissection and surgical control.


Assuntos
Hepatectomia/métodos , Neoplasias Hepáticas/cirurgia , Pré-Escolar , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Lactente , Neoplasias Hepáticas/diagnóstico , Imageamento por Ressonância Magnética , Masculino , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento
19.
BJU Int ; 97(2): 367-70, 2006 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-16430649

RESUMO

OBJECTIVE: To investigate lower urinary tract (LUT) functions in a prospective study in boys treated for posterior urethral distraction (PUD), as posterior urethral stricture, erectile dysfunction and incontinence can occur after various treatments for this rare injury in children. PATIENTS AND METHODS: Eleven boys were treated for PUD and resultant stricture between 1980 and 2000. Their age, the cause of trauma, extent of injury, previous treatment, and continence status were evaluated, and a urodynamic study conducted. Controls were 12 age-matched males who underwent similar cystometrography (CMG) and uroflowmetry. RESULTS: The mean (SD) age at injury was 8 (4) years and the cause was traffic accident in nine and crush injury in two boys. Seven boys had an isolated urethral rupture, two also had a bladder neck injury and two also had a perforated bladder. No gross neurological impairment developed after trauma. Voiding cysto-urethrograms after initial therapy showed vesico-ureteric reflux in five boys, but in only one at the time of the urodynamic studies. At the time of urodynamic study, the mean (SD) age of the patients was 15 (6) years; seven were fully continent, one had intermittent leakage, two were incontinent, and one had nocturnal enuresis. CMG-electromyography (EMG) showed a reduced maximum cystometric capacity in nine patients, reduced compliance in 10, stable detrusor in 11, synergic detrusor-sphincter activity in 11, and residual urine in one. The uroflowmetry-EMG study showed prolonged voiding time and flow time, decreased maximum flow urinary rate (Q(max)) and mean flow rate (Q(avg)). The shape of the flow curve showed an uninterrupted low-amplitude pattern. Comparing these patients with age-matched controls, CMG and uroflowmetry studies showed that the maximum cystometric bladder capacity, compliance, Q(max) and Q(avg) were all significantly lower in patients with PUD, while voiding time and flow time were significantly higher. CONCLUSION: The LUT deteriorates after treating PUD in boys. CMG and uroflowmetry findings are consistent with partial anatomical obstruction distal to the bladder. Our results are preliminary and full urodynamics, including pressure-flow studies, will be useful to support this conclusion. A urodynamic study should be integral in the management of PUD.


Assuntos
Uretra/lesões , Bexiga Urinária/lesões , Urodinâmica/fisiologia , Procedimentos Cirúrgicos Urológicos Masculinos/métodos , Adolescente , Criança , Pré-Escolar , Disfunção Erétil/etiologia , Humanos , Masculino , Estudos Prospectivos , Estatísticas não Paramétricas , Uretra/cirurgia , Obstrução Uretral/etiologia , Bexiga Urinária/cirurgia , Incontinência Urinária/etiologia
20.
Pediatr Surg Int ; 22(2): 135-9, 2006 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-16328338

RESUMO

Chest wall tumors (CWT) are rarely seen in childhood and surgery constitutes a complementary part of the therapy. The early and late results of CWT resection and chest wall reconstruction were evaluated retrospectively. The children who underwent chest wall resection for CWT between January 1990 and November 2003 were evaluated retrospectively. Seventeen children (male/female = 12/5, mean age: 7.58 years) underwent chest wall resection for CWT. Fifteen patients underwent initial biopsy (tru-cut, n = 8 or open biopsy, n = 7) and two underwent initial resection. The diagnosis was malignant tumor in 12 (70%) and benign in 5 (30%). They were Ewing's sarcoma (ES) (n = 4), primitive neuroectodermal tumor (PNET) (n = 3), Askin's tumor (n = 1), rhabdomyosarcoma (RMS) (n = 2), neuroblastoma (n = 2), osteochondroma (n = 1), aneurysmal bone cyst (n = 2) and hamartoma (n = 2). Preoperative chemotherapy was given to most patients with malignant tumor. All patients had only local tumor at the time of resection. Thoracotomy was performed in all patients. All tumor tissues with the affected rib/ribs were resected en bloc with the adjacent tissues. The number of resected ribs was 1 (n = 6), 2 (n = 7) and 3 (n = 4). Chest wall defects were repaired primarily (n = 8) or with grafts (n = 9). Dura (n = 4), Neuro-patch (n = 3) and Goretex (n = 2) were used for closure. Wound infection and pleural fistula occurred in one patient. Patients with benign tumor were free of complaints or complications during follow up. All patients with malignant tumor received postoperative chemotherapy. Local recurrence did not occur in all patients. Five patients developed distant metastasis and two died. Scoliosis was encountered in one patient during follow-up. Since most of the CWT are malignant and not initially suitable for surgical excision, the management includes tissue diagnosis either by tru-cut or open biopsy. Determination of malignant condition should be followed by an intensive chemotherapy. Chest wall resection is planned to control local disease. Chest wall reconstruction may be needed for large defects following resection of CWT. Prosthetic materials can be used safely. Early complications of the surgery are limited. The patients should be closely followed up for late complications such as scoliosis, restrictive pulmonary disease and for the development of metastasis, which is a part of natural course of malignant CWT in children.


Assuntos
Neoplasias Torácicas/cirurgia , Parede Torácica , Adolescente , Quimioterapia Adjuvante , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Terapia Neoadjuvante , Procedimentos de Cirurgia Plástica , Estudos Retrospectivos , Neoplasias Torácicas/patologia
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