Major discrepancy between clinical diagnosis of death and anatomopathological findings in adolescents with chronic diseases during 18-years.

OBJECTIVES: To evaluate the inconsistency between clinical diagnosis of death and autopsy findings in adolescents with chronic diseases. METHODS: A cross-sectional study including a sample of adolescents' autopsies who died in a pediatric and adolescent tertiary hospital over 18 consecutive years. During this period, there were n = 2912 deaths, and n = 581/2912(20%) occurred in adolescents. Of these, n = 85/581(15%) underwent autopsies and were analyzed. Further results were divided into two groups: Goldman classes I or II (high disagreement between main clinical diagnosis of death and anatomopathological findings, n = 26) and Goldman classes III, IV or V (low or no disagreement between these two parameters, n = 59). RESULTS: Median age at death (13.5 [10‒19] vs. 13 [10‒19] years, p = 0.495) and disease duration (22 [0‒164] vs. 20 [0‒200] months, p = 0.931), and frequencies for males (58% vs. 44%, p = 0.247) were similar between class I/II vs. class III/IV/V. The frequency of pneumonia (73% vs. 48%, p = 0.029), pulmonary abscess (12% vs. 0%, p = 0.026), as well as isolation of yeast (27% vs. 5%, p = 0.008), and virus (15% vs. 2%, p = 0.029) identified in the autopsy, were significantly higher in adolescents with Goldman class I/II compared to those with Goldman class III/IV/V. In contrast, cerebral edema was significantly lower in adolescents of the first group (4% vs. 25%, p = 0.018). CONCLUSION: This study showed that 30% of the adolescents with chronic diseases had major discrepancies between clinical diagnosis of death and autopsy findings. Pneumonia, pulmonary abscess, as well as isolation of yeast and virus were more frequently identified at autopsy findings in the groups with major discrepancies.

Major discrepancy between clinical diagnosis of death and anatomopathological findings in adolescents with chronic diseases during 18-years

Abstract Objectives: To evaluate the inconsistency between clinical diagnosis of death and autopsy findings in adolescents with chronic diseases. Methods: A cross-sectional study including a sample of adolescents' autopsies who died in a pediatric and adolescent tertiary hospital over 18 consecutive years. During this period, there were n = 2912 deaths, and n = 581/2912(20%) occurred in adolescents. Of these, n = 85/581(15%) underwent autopsies and were analyzed. Further results were divided into two groups: Goldman classes I or II (high disagreement between main clinical diagnosis of death and anatomopathological findings, n = 26) and Goldman classes III, IV or V (low or no disagreement between these two parameters, n = 59). Results: Median age at death (13.5 [10‒19] vs. 13 [10‒19] years, p = 0.495) and disease duration (22 [0‒164] vs. 20 [0‒200] months, p = 0.931), and frequencies for males (58% vs. 44%, p = 0.247) were similar between class I/II vs. class III/IV/V. The frequency of pneumonia (73% vs. 48%, p = 0.029), pulmonary abscess (12% vs. 0%, p = 0.026), as well as isolation of yeast (27% vs. 5%, p = 0.008), and virus (15% vs. 2%, p = 0.029) identified in the autopsy, were significantly higher in adolescents with Goldman class I/II compared to those with Goldman class III/IV/V. In contrast, cerebral edema was significantly lower in adolescents of the first group (4% vs. 25%, p = 0.018). Conclusion: This study showed that 30% of the adolescents with chronic diseases had major discrepancies between clinical diagnosis of death and autopsy findings. Pneumonia, pulmonary abscess, as well as isolation of yeast and virus were more frequently identified at autopsy findings in the groups with major discrepancies.

Persistent symptoms and decreased health-related quality of life after symptomatic pediatric COVID-19: A prospective study in a Latin American tertiary hospital

OBJECTIVES: To prospectively evaluate demographic, anthropometric and health-related quality of life (HRQoL) in pediatric patients with laboratory-confirmed coronavirus disease 2019 (COVID-19) METHODS: This was a longitudinal observational study of surviving pediatric post-COVID-19 patients (n=53) and pediatric subjects without laboratory-confirmed COVID-19 included as controls (n=52) was performed. RESULTS: The median duration between COVID-19 diagnosis (n=53) and follow-up was 4.4 months (0.8-10.7). Twenty-three of 53 (43%) patients reported at least one persistent symptom at the longitudinal follow-up visit and 12/53 (23%) had long COVID-19, with at least one symptom lasting for >12 weeks. The most frequently reported symptoms at the longitudinal follow-up visit were headache (19%), severe recurrent headache (9%), tiredness (9%), dyspnea (8%), and concentration difficulty (4%). At the longitudinal follow-up visit, the frequencies of anemia (11% versus 0%, p=0.030), lymphopenia (42% versus 18%, p=0.020), C-reactive protein level of >30 mg/L (35% versus 0%, p=0.0001), and D-dimer level of >1000 ng/mL (43% versus 6%, p=0.0004) significantly reduced compared with baseline values. Chest X-ray abnormalities (11% versus 2%, p=0.178) and cardiac alterations on echocardiogram (33% versus 22%, p=0.462) were similar at both visits. Comparison of characteristic data between patients with COVID-19 at the longitudinal follow-up visit and controls showed similar age (p=0.962), proportion of male sex (p=0.907), ethnicity (p=0.566), family minimum monthly wage (p=0.664), body mass index (p=0.601), and pediatric pre-existing chronic conditions (p=1.000). The Pediatric Quality of Live Inventory 4.0 scores, median physical score (69 [0-100] versus 81 [34-100], p=0.012), and school score (60 [15-100] versus 70 [15-95], p=0.028) were significantly lower in pediatric patients with COVID-19 at the longitudinal follow-up visit than in controls. CONCLUSIONS: Pediatric patients with COVID-19 showed a longitudinal impact on HRQoL parameters, particularly in physical/school domains, reinforcing the need for a prospective multidisciplinary approach for these patients. These data highlight the importance of closer monitoring of children and adolescents by the clinical team after COVID-19.

Benefits of the intermittent use of 6-mercaptopurine and methotrexate in maintenance treatment for low-risk acute lymphoblastic leukemia in children: randomized trial from the Brazilian Childhood Cooperative Group--protocol ALL-99.

PURPOSE To describe event-free survival (EFS) and toxicities in children with low-risk acute lymphoblastic leukemia (ALL) assigned to receive either continuous 6-mercaptopurine (6-MP) and weekly methotrexate (MTX) or intermittent 6-MP with intermediate-dose MTX, as maintenance treatment. PATIENTS AND METHODS Between October 1, 2000, and December 31, 2007, 635 patients with low-risk ALL were enrolled onto Brazilian Childhood Cooperative Group for ALL Treatment (GBTLI) ALL-99 protocol. Eligible children (n = 544) were randomly allocated to receive either continuous 6-MP/MTX (group 1, n = 272) or intermittent 6-MP (100 mg/m(2)/d for 10 days, with 11 days resting) and MTX (200 mg/m(2) every 3 weeks; group 2, n = 272). RESULTS The 5-year overall survival (OS) and EFS were 92.5% +/- 1.5% SE and 83.6% +/- 2.1% SE, respectively. According to maintenance regimen, the OS was 91.4% +/- 2.2% SE (group 1) and 93.6% +/- 2.1% SE (group 2; P = .28) and EFS 80.9% +/- 3.2% SE (group 1) and 86.5% +/- 2.8% SE (group 2; P = .089). Remarkably, the intermittent regimen led to significantly higher EFS among boys (85.7% v 74.9% SE; P = .027), while no difference was seen for girls (87.0% v 88.8% SE; P = .78). Toxic episodes were recorded in 226 and 237 children, respectively. Grade 3 to 4 toxic events for groups 1 and 2 were, respectively, 273 and 166 for hepatic dysfunction (P = .002), and 772 and 636 for hematologic episodes (P = .005). Deaths on maintenance were: seven (group 1) and one (group 2). CONCLUSION The intermittent use of 6-MP and MTX in maintenance is a less toxic regimen, with a trend toward better long-term EFS. Boys treated with the intermittent schedule had significantly better EFS.

Intracaval and intracardiac extension of Wilms' tumor. The influence of preoperative chemotherapy on surgical morbidity.

OBJECTIVES: The aim of this retrospective study is to compare surgical complications and long-term survival in children with Wilms' tumor (WT) and tumor thrombus receiving or not preoperative chemotherapy. MATERIALS AND METHODS: Review of the charts of 155 children with WT treated between 1983 and 2005, and analysis of 16/155 (10.3%) children with WT who presented cavoatrial tumor extension, being 8/16 IVC and 8/16 atrial thrombus. RESULTS: Median age was 54 months. 2/16 had cardiac failure as the first symptom. 11/16(7 IVC and 4 atrial extension) (67%) were submitted to preoperative chemotherapy with vincristine plus actinomycin D, and 5/16(1 IVC and 4 atrial) (33%) underwent initial nephrectomy and thrombus resection. So, 11 patients were submitted to preoperative VCR/ACTD and 2/11 (18.1%) had complete regression of the thrombus, 6/11(54.5%) partial regression and 3/11 (27%) had no response. Among the partial responders, nephrectomy with thrombus removal was performed in all, including one patient with previous intracardiac involvement, without extracorporeal circulation procedures. In two of the three non-responders, cardiopulmonary bypass was necessary for thrombus removal. There were no surgical related deaths. Long-term survival is 91% in the group submitted to preoperative chemotherapy and 100% in the group who had surgery as first approach. CONCLUSION: Preoperative chemotherapy was able to reduce thrombus extension in 8/11 (73%) treated patients and cardiopulmonary bypass was avoided in 2 patients with atrial thrombus. Surgical resection of tumor and thrombus was successful in all cases, receiving or not preoperative chemotherapy and overall survival was similar in both groups.

Intracaval and intracardiac extension of Wilms' tumor: the influence of preoperative chemotherapy on surgical morbidity

OBJECTIVES: The aim of this retrospective study is to compare surgical complications and long-term survival in children with Wilms' tumor (WT) and tumor thrombus receiving or not preoperative chemotherapy MATERIALS AND METHODS: Review of the charts of 155 children with WT treated between 1983 and 2005, and analysis of 16/155 (10.3 percent) children with WT who presented cavoatrial tumor extension, being 8/16 IVC and 8/16 atrial thrombus RESULTS: Median age was 54 months. 2/16 had cardiac failure as the first symptom. 11/16(7 IVC and 4 atrial extension) (67 percent) were submitted to preoperative chemotherapy with vincristine plus actinomycin D, and 5/16(1 IVC and 4 atrial) (33 percent) underwent initial nephrectomy and thrombus resection. So, 11 patients were submitted to preoperative VCR/ACTD and 2/11 (18.1 percent) had complete regression of the thrombus, 6/11(54.5 percent) partial regression and 3/11 (27 percent) had no response. Among the partial responders, nephrectomy with thrombus removal was performed in all, including one patient with previous intracardiac involvement, without extracorporeal circulation procedures. In two of the three non-responders, cardiopulmonary bypass was necessary for thrombus removal. There were no surgical related deaths. Long-term survival is 91 percent in the group submitted to preoperative chemotherapy and 100 percent in the group who had surgery as first approach CONCLUSION: Preoperative chemotherapy was able to reduce thrombus extension in 8/11 (73 percent) treated patients and cardiopulmonary bypass was avoided in 2 patients with atrial thrombus. Surgical resection of tumor and thrombus was successful in all cases, receiving or not preoperative chemotherapy and overall survival was similar in both groups.

Measles and rubella antibody status in children after treatment for acute lymphoblastic leukemia

OBJETIVO: Avaliar a história vacinal e a situação da proteção vacinal contra sarampo e rubéola em crianças portadoras de leucemia linfóide aguda após o término do tratamento. MÉTODOS: O estado imunológico contra o sarampo e a rubéola foi avaliado pela técnica ELISA em 22 crianças com leucemia linfóide aguda após o término do tratamento. RESULTADOS: Dos 22 pacientes, 20 haviam recebido previamente duas doses da vacina do sarampo, e 18 deles, uma dose da vacina da rubéola. Soropositivos para sarampo e rubéola resultaram em 65 e 88,9 por cento, respectivamente, sem correlação com idade do paciente, agressividade do tratamento ou tempo decorrido entre final do tratamento e coleta da amostra. CONCLUSÃO: Detectamos falha na proteção vacinal contra sarampo e rubéola em 35 e 11,1 por cento dos casos, respectivamente. Recomendamos, ao final do tratamento para leucemia linfóide aguda, aplicar reforço da vacina contra sarampo, avaliar o estado imunológico contra rubéola e, se necessário, revacinar o paciente.

OBJECTIVE: To assess the vaccination history and the status of vaccine-induced protection from measles and rubella in children after treatment for acute lymphoblastic leukemia. METHODS: Measles and rubella immunological status was assessed by the ELISA technique for 22 children previously treated for acute lymphoblastic leukemia. RESULTS: From the total of 22 patients, 20 had been given two doses of measles vaccine and 18 had had one dose of rubella vaccine. The percentage of patients seropositive for measles and rubella were 65 and 88.9 percent, respectively, with no correlation with age of patient, aggression of treatment or the time passed between the end of treatment and sample collection. CONCLUSIONS: We detected that vaccination had failed against measles and rubella in 35 and 11.1 percent of cases, respectively. We recommend that a measles booster be given after the completion of treatment for acute lymphoblastic leukemia and that rubella immunity status should be assessed at this point, with revaccination performed when necessary.

Measles and rubella antibody status in children after treatment for acute lymphoblastic leukemia.

OBJECTIVE: To assess the vaccination history and the status of vaccine-induced protection from measles and rubella in children after treatment for acute lymphoblastic leukemia. METHODS: Measles and rubella immunological status was assessed by the ELISA technique for 22 children previously treated for acute lymphoblastic leukemia. RESULTS: From the total of 22 patients, 20 had been given two doses of measles vaccine and 18 had had one dose of rubella vaccine. The percentage of patients seropositive for measles and rubella were 65 and 88.9%, respectively, with no correlation with age of patient, aggression of treatment or the time passed between the end of treatment and sample collection. CONCLUSIONS: We detected that vaccination had failed against measles and rubella in 35 and 11.1% of cases, respectively. We recommend that a measles booster be given after the completion of treatment for acute lymphoblastic leukemia and that rubella immunity status should be assessed at this point, with revaccination performed when necessary.

Further experience with laparoscopic nephrectomy for Wilms' tumour after chemotherapy.

OBJECTIVE: To report the results of laparoscopic nephrectomy for unilateral Wilms' tumour in children treated with chemotherapy before surgery. PATIENTS AND METHODS: Eight children with unilateral nonmetastatic Wilms' tumour included in the International Society of Pediatric Oncology 2001 protocol were treated with vincristine/actinomycin D and then had laparoscopic nephrectomy and lymph-node sampling. A Veress needle puncture was made and a four-trocar transperitoneal approach was used in all cases. The tumour was extracted with no morcellation through a Pfannenstiel incision. RESULTS: All eight tumours were completely removed, with lymph node samples; intraoperative bleeding was minimal (50 mL). There were no complications after surgery and patients were discharged after 2-3 days. No recurrences of disease, port-site implantation or long-term complications were detected. CONCLUSIONS: Laparoscopic nephrectomy for unilateral Wilms' tumour is feasible in children after chemotherapy; it is safe and allows the complete surgical approach required for treating this tumour. Although the patients had a good long-term follow-up, more patients are needed to compare the results of laparoscopic techniques with open surgery.

Ulcerated rhabdomyosarcoma as portal of entry for tetanus in a previously nonimmunized child.

Infectious diseases represent one of the most important secondary problems related to the treatment of childhood cancer, being the leading cause of death in this population. They are predominantly of bacterial and fungal etiology. The association between tetanus, a bacterial vaccine-preventable disease, and cancer is virtually undescribed. The authors present the case of a previously nonimmunized child, due to his parents' choice, who developed severe tetanus with an ulcerated rhabdomyosarcoma as portal of entry. Due to an unfavorable evolution, the child underwent a hip disarticulation to provide tetanus control. The ulterior tumor management was successful: the child has been off therapy for more than 108 months with no evidence of disease.

Neuroblastoma em adultos: relato de caso / Neuroblastoma in adults: case report

O neuroblastoma e o tumor extracraniano mais comum na infancia, sendo extremamente raro em adultos. Os autores relatam o caso de um homem de 28 anos com neuroblastoma, juntamente com revisao da literatura pertinente

A t(9; 11) translocation in childhood acute mixed leukemia

We present the case of a child with acute lymphoid teukemia (ALL) who was morphologically classified as FAB L1 (PAS and peroxidase were negative). Remission was achieved with an ALL-type protocol (GBTLI). Five months after the discontinuation of therapy, the patient presented mixed leukemia (CD10, CD19,CD13 and CD33 were positive) with (9;11) (p21;q23) translocation. Unfortunately, as cytogenetic and immunophenotype studies were not performed at diagnosis, two possibilities could be considered for the relapse; secondarly mixed leukemia with clonal chromosome changes, or mixed leukemia from the beginning.