Pulsed Radiofrequency Rhizotomy of the Genicular Nerves of the Knee Guided by Radioscopy and Ultrasonography: Step-By-Step Technique.

Osteoarthritis (OA) of the knee is highly prevalent and causes pain, stiffness, and harms the quality of life of millions of patients. Scientific evidence about radiofrequency ablation or rhizotomy of genicular nerves has been presented with increasing frequency in the literature for the treatment of chronic pain related to knee OA as an alternative to total knee arthroplasty. The main indication for this procedure is symptomatic OA unresponsive to conservative treatment, regardless of the disease evolution, although more common indications are in Kellgren-Lawrence grade III or IV, in post-total knee arthroplasty residual pain without an identified cause, in patients with comorbidities and high surgical risk, and those who do not want to undergo surgery. The aim of this study is to describe the step-by-step rhizotomy technique with pulsed radiofrequency of the 3 genicular nerves, guided by radioscopy and ultrasonography.

Treatment of Distal Patellar Tendon Chronic Rupture: The X-Wave Technique.

Injuries to the patellar tendon (PT) are associated with knee function deterioration and loss of the capacity to perform daily and sports activities. Patellar tendon injury is often misdiagnosed at emergency rooms, leading to chronic proximal retraction and a challenging clinical scenario. Proximal PT injuries are more common, while distal ones, which can involve tibial bone avulsion fractures or direct tendon avulsion, are rarer. The low incidence of distal PT rupture and the variety of injury patterns make a personal approach reasonable when based on the intraoperative findings and the surgeon's experience. Our purpose is to describe a surgical technique to restore the knee extensor mechanism after chronic distal PT rupture using two kinds of graft, one as a waveform augmentation of the native tendon and the other as reinforcement in a letter X aspect.

Positioning Technique of the Fixed Knee in Hyperflexion for the Transportal Femoral Tunnel During Reconstruction of the Anterior Cruciate Ligament.

During reconstruction surgery of the anterior cruciate ligament, the evolution of the femoral tunnel from the transtibial to the transportal path provides greater accuracy in reaching the desired anatomic point. However, there are also some new challenges, such as correct execution, reproducibility, and minimizing the risk of iatrogenic injury from its use. In an effort to overcome these challenges, we have proposed the use of a positioner, which was developed by our group and allows the leg to stay in the desired position, without variations in the operation or aid from a medical assistant. This manuscript presents our femoral tunnel preparation technique and its application in clinical practice.

Shorter Maintenance Therapy in Childhood Acute Lymphoblastic Leukemia: The Experience of the Prospective, Randomized Brazilian GBTLI ALL-93 Protocol.

AIM: Maintenance therapy is an important phase of the childhood ALL treatment, requiring 2-year long therapy adherence of the patients and families. Weekly methotrexate with daily 6-mercaptopurine (6MP) constitutes the backbone of maintenance therapy. Reduction in the maintenance therapy could overweight problems related with poverty of children with ALL living in limited-income countries (LIC). OBJECTIVE: To compare, prospectively, the EFS rates of children with ALL treated according to two maintenance regimens: 18 vs. 24 months duration. MATERIALS AND METHODS: From October 1993 to September 1999, 867 consecutive untreated ALL patients <18 years of age were treated according to the Brazilian Cooperative Group for Childhood ALL Treatment (GBTLI) ALL-93 protocol. Risk classification was based exclusively on patient's age and leukocyte count (NCI risk group) and clinical extra medullary involvement of the disease. Data were analyzed by the intention-to-treat approach. RESULTS: Fourteen patients (1.6%) were excluded: wrong diagnosis (n = 7) and previous corticosteroid (n = 7). Of the 853 eligible patients, 421 were randomly allocated, at study enrollment, to receive 18-month (group 1) and 432 to receive 24-month (group 2) maintenance therapy. Complete remission rate was achieved in 96% of the patients (817/853). Twenty-eight patients (3.4%) died during the induction phase. Thirty-four patients (4.0%) were lost to follow-up. The overall EFS was 66.1 ± 1.7% at 15 years. No difference was seen according to maintenance: EFS15y was 65.8 ± 2.3% (group 1) and 66.3 ± 2.3% (group 2; p = 0.79). No difference between regimens was detected after stratifying the analyses according to factors associated with adverse prognosis in this study (age group <1 year or >10 years and high WBC at diagnosis). Overall death in remission rate was 6.85% (56 patients). Deaths during maintenance were 13 in group 1 and 12 in group 2, all due to infection. Over 15 years of follow-up, two patients both from group 2 presented a second malignancy (Hodgkin's disease and thyroid carcinoma) after 8.3 and 11 years off therapy, respectively. CONCLUSION: Six-month reduction of maintenance therapy in ALL children treated according to the GBTLI ALL-93 protocol provided the same overall outcome as 2-year duration regimen.

Clinical and molecular epidemiology of neonatal leukemia in Brazil.

The clinical and molecular findings of 77 cases of neonatal leukemia (NL) and 380 of infant leukemia (IL) were selected to distinguish features between NL and IL. Somatic gene mutations associated with acute leukemia including FLT3, RAS and PTPN11 were revisited. There were 42 cases of congenital leukemia associated with Down syndrome (DS) and 39 of these cases presented features of acute myeloid leukemia (AML)-M7. Twenty-seven of the DS cases underwent spontaneous remission and were reclassified as a transient myeloproliferative disorder. GATA1 mutations were found in 70% of these cases. In non-DS, frequent abnormalities were MLL rearrangements, mainly MLL-AFF1 in acute lymphoblastic leukemia and MLL-MLLT3 in AML. The FLT3 mutation was not found, while RAS (n = 4) and PTPN11 (n = 2) mutations were identified and reported for the first time in NL. There was substantial evidence to support that somatic abnormalities occur in utero. Thus, congenital leukemia is a good model for understanding leukemogenesis.

Contribution of multiparameter flow cytometry immunophenotyping to the diagnostic screening and classification of pediatric cancer.

Pediatric cancer is a relatively rare and heterogeneous group of hematological and non-hematological malignancies which require multiple procedures for its diagnostic screening and classification. Until now, flow cytometry (FC) has not been systematically applied to the diagnostic work-up of such malignancies, particularly for solid tumors. Here we evaluated a FC panel of markers for the diagnostic screening of pediatric cancer and further classification of pediatric solid tumors. The proposed strategy aims at the differential diagnosis between tumoral vs. reactive samples, and hematological vs. non-hematological malignancies, and the subclassification of solid tumors. In total, 52 samples from 40 patients suspicious of containing tumor cells were analyzed by FC in parallel to conventional diagnostic procedures. The overall concordance rate between both approaches was of 96% (50/52 diagnostic samples), with 100% agreement for all reactive/inflammatory and non-infiltrated samples as well as for those corresponding to solid tumors (n = 35), with only two false negative cases diagnosed with Hodgkin lymphoma and anaplastic lymphoma, respectively. Moreover, clear discrimination between samples infiltrated by hematopoietic vs. non-hematopoietic tumor cells was systematically achieved. Distinct subtypes of solid tumors showed different protein expression profiles, allowing for the differential diagnosis of neuroblastoma (CD56(hi)/GD2(+)/CD81(hi)), primitive neuroectodermal tumors (CD271(hi)/CD99(+)), Wilms tumors (>1 cell population), rhabdomyosarcoma (nuMYOD1(+)/numyogenin(+)), carcinomas (CD45(-)/EpCAM(+)), germ cell tumors (CD56(+)/CD45(-)/NG2(+)/CD10(+)) and eventually also hemangiopericytomas (CD45(-)/CD34(+)). In summary, our results show that multiparameter FC provides fast and useful complementary data to routine histopathology for the diagnostic screening and classification of pediatric cancer.

Detection of mutations in GATA1 gene using automated denaturing high-performance liquid chromatography and direct sequencing in children with Down syndrome.

Denaturing high-performance liquid chromatography (dHPLC) was developed to screen DNA variations by separating heteroduplex and homoduplex DNA fragments by ion-pair reverse-phase liquid chromatography. In this study, we have evaluated the dHPLC screening method and direct sequencing for the detection of GATA1 mutations in peripheral blood and bone marrow aspirates samples from children with Down syndrome (DS). Cases were ascertained consecutively as part of an epidemiological study of DS and hematological disorders in Brazil. A total of 130 samples corresponding to 115 children with DS were analysed using dHPLC and direct sequencing methods to detect mutations in GATA1 exons 2, 3 and 4 gene sequences. The overall detection rate of sequencing and dHPLC screening methods was similar. Twenty mutations were detected in exon 2 and one mutation in exon 3 (c.231_232 dupGT) sequences of acute megakaryoblastic leukemia and transient leukemia samples. Four GATA1 mutations were newly described [c.155C > G; c.156_178 del23 bp; c.29_30 del GG; c.182C > A and c.151A > T,c.153_162 del 10 bp). Out of four, three had single nucleotide change. In conclusion, our results indicate that dHPLC is an efficient and valuable tool for GATA1 mutational analysis.

Linfoma de Burkitt com envolvimento focal do centro germinativo simulado hiperplasia linfóide reacional / Burkitt's lymphoma with focal involvement of germinal center simulating lymphoid hyperplasia

O desenvolvimento do centro germinativo pelo linfoma de Burkitt cria dificuldades diagnósticas de grande conseqüência clínica. Descrevemos o caso de uma criança do sexo masculino com nove meses de idade, com massa tumoral osteilítica localizada na mandíbula. Ao exame físico, gânglio cervical aumentado, próximo à massa. Gânglios paravertebrais aumentados foram observados através de tomografia computadorizada. A biopsia do gânglio paravertebrais aumentados foram observados através de tomografia computadorizada. A biópsia do gânglio cervical mostrou centros germinativos hiperplásicos ao lado de outros parcial ou totalmente substituídos pelo linfoma. O aspecto de "céu estrelado" simulava, ao pequeno aumento, uma hiperplasia linfóide reacional. O estudo dos diversos padröes de centro germinativo foi essencial para o diagnóstico. Exame imuno-histoquímico mostrou células neoplásicas CD20+; a hibridizaçäo in situ para a presença de EBV foi negativa

Retinoblastoma: estudo retrospectivo de 22 casos / Retinoblastoma: retrospective study of 22 cases

Revisou-se os prontuários dos pacientes com retinoblastoma referidos ao setor de Onco-hematologia Pediátrica do Hospital dos Servidores do Estado - RJ no período de 1989 a 1995, numtotal de 22 casos. Realizou-se um estudo epidemiológico onde foram avaliados: a proporçäo de sexos e de raças, a uni ou bilateralidade do tumor e seu estadiamento histopatológico e a sobrevida em relaçäo ao esquema terapêutico adotado. Os dados obtidos pela nossa casuística revelam que o retinoblastoma, em nosso meio, continua sendo uma doença de diagnóstico tardio, o que eleva sobremaneira a taxa de mortalidade entre os pacientes afetados por este tumor

Efeitos tardios do tratamento da Doença de Hodgkin em crianças abaixo de 12 anos de idade / Late effects of treatment for Hodgkin's disease in children below 12 years of age

A doença de Hodgkin é um tipo de neoplasia maligna que apresenta aspectos epidemiológicos particularmente interessantes, tornando-a mais frequente em crianças nos paises em desenvolvimento. Sua cura no últimos 30 anos tem sido elevada a patamares muito altos com o emprego de radioterapia e quimioterapia. Com o objetivo de avaliar os efeitos indesejáveis tardios do tratamento, 24 crianças com idades inferiores a 12 anos ao diagnóstico (20 meninos e 4 meninas), tratadas em uma única instituição (Hospital dos Servidores do Estado - RJ), entre maio de 1979 e fecereiro de 1994, foram avaliadas...Concluímos que o emprego de radioterapia nesta faixa etária deve se limitar às grandes massas tumorais e em doses inferiores a 25 Gy. Radioterapia abdominal somente deverá ser substituída devido ao seu elevado potencial carcinogênico. É também objetivo deste trabalho alertar os serviços que tratam deste tipo de patologia no nosso país par falhas que podem ser cometidas e que poderão trazer graves consequências para p futuros destas crianças

Efeitos tardios do tratamento da doença de Hodgkin em crianças abaixo de 12 anos de idade

A doença de Hodgkin é um tipo de neoplasia maligna que apresenta aspectos epidemiológicos particularmente interessantes, tomando-a mais freqüente em crianças nos países em desenvolvimento. Sua cura nos últimos 30 anos, tem sido elevada a patamares muito altos com o emprego de radioterapia e quimioterapia. Com objetivo de avaliar os efeitos indesejáveis tardios do tratamento, 24 crianças com idades inferiores a 12 anos ao diagnóstico ( 20 meninos e 4 meninas ), tratadas em uma única instituição (Hospital dos Servidores do Estado-RJ), entre maio de 1979 e fevereiro de 1994, foram avaliadas. A sobrevida do grupo foi de 80% (m=6 anos). Vinte crianças foram submetidas a tratamento misto radio-quimioterápico e 4 pacientes a tratamento apehas quimioterápico. A função cardíaca foi avaliada através de ecocardiograma bi-dimensional; a função pulmonar (VEF1 e CV) foi verificada através de espirômetro de fluxo; espermograma foi solicitado aos meninos púberes, além da dosagem de FSH, LH e testosterona, para avaliar a função gonadal. Nas meninas também foi dosada FSH e LH; a função tireoidiana através da dosagem de T3,T4,T4 livre e TSH foi realizada em todos os pacientes submetidos a radioterapia em região próxima a tireóide. Os exames foram realizados em média 9,1 anos (3 a 18 anos) após o tratamento. Observou-se ainda a incidência de neoplasias secundárias no grupo. Dos 24 pacientes tratados, 4 faleceram ( 3 de doença progressiva) e 2 se encontram fora de acompanhamento. Apenas 1/11 pacientes estudados, e que havia feito uso de antraciclinas + radioterapia de mediastino apresentou fração de encurtamento do ventriculo esquerdo de 24% ( % DLVD), embora permanecesse assintomático. Um de 12 pacientes submetidos a radioterapia em região próxima a tireóide, apresentou hipotireoidismo incipiente, com níveis elevados de TSH. Dois pacientes apresentaram hipertireoidismo clínico. Todos os meninos estudados através de espermograma ( 8 pacientes) apresentaram resultados anormais (azoospermia em 6 e oligospermia em 2). A dosagem do FSH esteve elevada em 6/8 pacientes e o LH em 2/8 pacientes. Uma das meninas submetida a radioterapia abdominal desenvolveu amenorréia primária. Alteração da função pulmonar não foi observada em nenhum paciente. Um menino apresentou osteossarcoma de ilíaco em área irradiada, cerca de 10 anos após o tratamento. Concluimos que o emprego de radioterapia nesta faixa etária deve se limitar às grandes massa tumorais, e em doses inferiores a 25 Gy. Radioterapia abdominal somente deverá ser realizada com a proteção das gônadas, tanto masculinas quanto femininas. A procarbazina não deve ser utilizada nos meninos. A mecloretamina deverá ser substituída devido ao seu elevado potencial carcinogênico. É também objetivo deste trabalho alertar os serviços que tratam deste tipo de patologia no nosso país para falhas que podem ser cometidas e que poderão trazer graves conseqüências para o futuro destas crianças

Hodgkin's disease is a kind of malignancy with epidemiological features that are particularly interesting, as it is more frequent in children in developing countries. During the past 30 years, its cure has reached very high rates with the introduction of radiation therapy and chemotherapy. Based upon our objective to assess late untoward effects of treatment, we studied 24 children below 12 years old ( twenty boys and 4 girls) treated in a single center ( Hospital dos Servidores do Estado-RJ ) from May 1979 through February 1994. The group's survival rate was 80% ( m= 6 yr. ). Twenty children underwent mixed therapy with radiation and chemotherapy, and 4 patients received chemotherapy only. Cardiac function was assessed through two-dimensional echocardiography; pulmonary function ( FEV1 and VC ) was measured with flow spirometry; sperm counts were obtained for male adolescents, as well as determination of FSH, LH, and testosterone, in order to assess gonadal function. For girls, FSH and LH were also determined. Thyroid function was studied through T3, T4, free T4, and TSH measurements in all patients who were treated with radiation therapy to a field close to the thyroid gland. All tests were done on average 9.1 years ( three to 18 years) after treatment. In adition, the group was studied regarding incidence of secondary malignancies. Among 24 treated patients, 4 died ( three of progressive disease ) and two were lost to follow-up. Only 1 of 11 studied patients, who have received anthracycline plus radiation therapy to the mediastinum, showed a left ventricular shortening fraction of 24% ( % AL VD ) , although he remained asymptomatic. Of 12 patients who underwent radiation therapy to a field close to the thyroid gland, one only presented with compensated hypothyroidism, showing increased TSH levels. Two patients developed clinically apparent hyperthyroidism. All boys who were studied through sperm count ( 8 patients ) showed abnormal results ( azoospermia in 6 and oligospermia in 2 ). FSH level was increased in 6/8 patients, and LH level in 2/8 patients. One of the girls treated with abdominal radiation therapy developed primary amenorrhea. Pulmonary function abnormalities were found in no patients. A boy developed an osteosarcoma in os ilium within a radiated field, approximately 10 years after treatment. We conclude that radiation therapy should be restricted in this age group to large neoplastic masses, and in dosages below 25 Gy. Abdominal radiation therapy should be instituted only with gonad shields placed for both sexes. Procarbazine should not be used in boys. Mechlorethamine gBBulll be fep}neoo due to itg high C1lfcinogenic potential. Another objective of thig Qtudy is to warn all centers caring for children with such neoplasm in our country, emphasising the errors that can be made and which will bring serious consequences to the lives of affected children