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1.
Eur J Pediatr ; 2019 Oct 17.
Artigo em Inglês | MEDLINE | ID: mdl-31624947

RESUMO

Life-limiting conditions in children in specialized pediatric palliative care (PPC) are manifold. The "Together for Short Lives" (TfSL) association established four disease categories, which represent the most common illness trajectories. Better understanding the palliative care needs and symptoms of children within these TfSL groups will result in improved anticipation of clinical problems and tailored care. During this retrospective single-center cohort study, 198 children, adolescents, and young adults (CAYAs) were in PPC. Mean age at referral was 8.7 years (range 0.0-25.0), mean duration of care 355 days (range 1-2754). One hundred six (53.5%) CAYAs died during the study period. Sixty-five (32.8%) CAYAs were assigned to TfSL-1, 13 (6.6%) to TfSL-2, 49 (24.7%) to TfSL-3, and 71 (35.9%) to TfSL-4. Home visits were conducted on average every 9.6 days in TfSL-1, 18.9 days in TfSL-2, 31.7 days in TfSL-3, and 31.8 days in TfSL-4 (p value < 0.01).Conclusions: Intensity of palliative care significantly differed between the TfSL groups. Neurological and gastrointestinal symptoms were most prominent across all TfSL groups. Symptom cluster analysis showed distinct clusters in TfSL-1 (cluster 1, fatigue/lack of appetite/nausea/somnolence; cluster 2, dyspnea/fear/myoclonus/seizures/spasticity) and TfSL-3/4 (cluster 1, spasticity; cluster 2, all other symptoms). What is Known: • The four TfSL (together for short lives) groups represent the four most common illness trajectories of pediatric palliative care patients. • Better understanding the palliative care needs and symptoms of children within these four TfSL groups will result in improved anticipation of clinical problems and tailored care. What is New: • In our study, TfSL-1 represented the largest individual group of patients, also requiring the most intensive care (defined by the number of visits per days of care). • Symptom cluster analysis revealed distinct symptom clusters in TfSL-1 and TfSL-3/4, which can be used to anticipate clinically common challenges in these patients.

3.
Orphanet J Rare Dis ; 13(1): 112, 2018 Jul 09.
Artigo em Inglês | MEDLINE | ID: mdl-29986738

RESUMO

BACKGROUND: Our objective was to evaluate children with metabolic diseases in paediatric palliative home care (PPC) and the process of decision-making. This study was conducted as single-centre retrospective cohort study of patients in the care of a large specialized PPC team. RESULTS: Between 01/2013 and 09/2016, 198 children, adolescents and young adults were in the care of our PPC team. Twenty-nine (14.6%) of these patients had metabolic conditions. Median age at referral was 2.6 years (0-24), median duration of care 352 days (3-2248) and median number of home visits 13 (1-80). Most patients are still alive (16; 55.2%). Median number of drugs administered was 5 (range 0-12), antiepileptics were given most frequently. Symptom burden was high in all children with metabolic disorders at referral and remained high throughout care. Predominant symptoms were gastrointestinal, respiratory and neurologic symptoms. Children with metabolic conditions, who were referred to PPC younger than 1 year of age had a shorter period of care and died earlier compared to those children, who were referred to PPC later in their lives (older than 10 years of age). Eleven (37.9%) of the children initially had no resuscitation restrictions and 7 (53.8%) of those who died, did so on ICU. CONCLUSIONS: About 15% of children with life-limiting conditions in PPC present with metabolic diseases. Symptom burden is high with neurologic, respiratory and gastrointestinal symptoms being the most frequent and most of those being difficult to treat. In these children, particular attention needs to be addressed to advance care planning.

4.
Oncotarget ; 9(25): 17867-17875, 2018 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-29707152

RESUMO

Pediatric advance care planning seeks to ensure end-of-life care conforming to the patients/their families' preferences. To expand our knowledge of advance care planning and "medical orders for life-sustaining treatment" (MOLST) in pediatric palliative home care, we determined the number of patients with MOLST, compared MOLST between the four "Together for Short Lives" (TfSL) groups and analyzed, whether there was a relationship between the content of the MOLST and the patients' places of death. The study was conducted as a single-center retrospective analysis of all patients of a large specialized pediatric palliative home care team (01/2013-09/2016). MOLST were available in 179/198 children (90.4%). Most parents decided fast on MOLST, 99 (55.3%) at initiation of pediatric palliative home care, 150 (83.4%) within the first 100 days. MOLST were only changed in 7.8%. Eighty/179 (44.7%) patients decided on a Do Not Attempt Cardio-Pulmonary Resuscitation (DNACPR) order, 58 (32.4%) on treatment limitations of some kind and 41 (22.9%) wished for the entire spectrum of life-sustaining measures (Full Code). Most TfSL group 1 families wanted DNACPR and most TfSL group 3/4 parents Full Code. The majority (84.9%) of all DNACPR patients died at home/hospice. Conversely, all Full Code patients died in hospital (80% in an intensive care setting). The circumstances of the childrens' deaths can therefore be predicted considering the content of the MOLST. Regular advance care planning discussions are thus a very important aspect of pediatric palliative home care.

5.
Oncotarget ; 8(52): 89939-89948, 2017 Oct 27.
Artigo em Inglês | MEDLINE | ID: mdl-29163800

RESUMO

Introduction: Hematologic malignancies (HM) represent the most common neoplasms in childhood. Despite improved overall survival rates, they are still a major contributor to cancer death in children. Aims: To determine the proportion of children with HM in pediatric palliative care (PPC) and to identify the clinical characteristics and symptoms in comparison to children with extracranial solid tumors (non HM patients). Patients and Methods: This study was conducted as a single-center retrospective cohort study of patients in the care of a large specialized PPC team. Results: Fifteen HM and 50 non HM patients were included. Symptoms in which HM patients scored significantly higher than non HM patients were mucositis, difficulty moving, somnolence, fatigue, petechiae and paleness. Blood transfusions were more frequently administered to HM patients, but large external hemorrhage was not observed in any child. A large variety of drugs and appliances were needed by the patients, with morphine being the most frequently prescribed drug. During the study period, a much larger and over the years even increasing number of HM patients (not in the care of the PPC team) died in hospital with an (assumed) curative intent, with two thirds dying in the ICU. Conclusions: Children with HM were referred to outpatient PPC with almost the full clinical picture of advanced leukemia. Noteworthy, the number of children with HM dying at home is decreasing in our center, instead a substantial proportion received high-intensity medical hospital care including novel anticancer therapies. These patients thus seem to be at an increased risk of dying in hospital as the right time to transfer them to palliative care is oftentimes missed.

6.
Eur J Pediatr ; 176(10): 1319-1327, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28808789

RESUMO

The number of children without a diagnosis in pediatric palliative home care and the process of decision-making in these children are widely unknown. The study was conducted as single-center retrospective cohort study. Between January 2013 and September 2016, 198 children and young adults were cared for; 27 (13.6%) of these were without a clear diagnosis at the start of pediatric palliative home care. A definite diagnosis was ultimately achieved in three children. Median age was 7 years (0-25), duration of care 569 days (2-2638), and number of home visits 7.5 (2-46). Most patients are still alive (19; 70.4%). Median number of drugs administered was eight (range 2-19); antiepileptics were given most frequently. Despite the lack of a clear diagnosis (and thus prognosis), 13 (48.1%) parents faced with their critically ill and clinically deteriorating children decided in favor of a DNAR order. Comparing this with 15 brain-injured children, signs, symptoms, and supportive needs were similar in both groups. CONCLUSION: Children without a clear diagnosis are relatively common in pediatric palliative care and have-like all other patients-the right to receive optimized and symptom-adapted palliative care. Parents are less likely to choose treatment limitation for children who lack a definitive diagnosis. What is Known: • A clear diagnosis is usually considered important for best-practice pediatric palliative care (PPC) including advanced care planning (ACP). • Timely initiation of pediatric palliative care (PPC) is highly recommended in children with life-limiting conditions. What is New: • SWAN (syndrome without a name) children show similar signs and symptoms (mostly neurological) and have similar supportive needs as brain-injured children. • Defining treatment limitations in advance care planning is more difficult for parents of SWAN compared to brain-injured children.


Assuntos
Diagnóstico Tardio/estatística & dados numéricos , Cuidados Paliativos , Adolescente , Planejamento Antecipado de Cuidados/ética , Criança , Pré-Escolar , Tomada de Decisão Clínica , Tomada de Decisões , Diagnóstico Tardio/ética , Diagnóstico Tardio/psicologia , Feminino , Alemanha , Humanos , Lactente , Recém-Nascido , Masculino , Cuidados Paliativos/ética , Cuidados Paliativos/métodos , Cuidados Paliativos/psicologia , Cuidados Paliativos/estatística & dados numéricos , Pais/psicologia , Relações Profissional-Família , Estudos Retrospectivos , Síndrome , Assistência Terminal/ética , Assistência Terminal/métodos , Assistência Terminal/psicologia , Suspensão de Tratamento/ética , Adulto Jovem
7.
Eur J Paediatr Neurol ; 20(6): 898-903, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27506815

RESUMO

BACKGROUND: Cannabis extracts have a wide therapeutic potential but in many countries they have not been approved for treatment in children so far. OBJECTIVE: We conducted an open, uncontrolled, retrospective study on the administration of dronabinol to determine the value, efficacy, and safety of cannabis-based medicines in the treatment of refractory spasticity in pediatric palliative care. DESIGN AND PARTICIPANTS: Sixteen children, adolescents and young adults having complex neurological conditions with spasticity (aged 1.3-26.6 years, median 12.7 years) were treated with dronabinol by our specialized pediatric palliative care team between 01.12.2010 and 30.04.2015 in a home-care setting. Therapeutic efficacy and side effects were closely monitored. RESULTS: Drops of the 2.5% oily tetrahydrocannabinol solution (dronabinol) were administered. A promising therapeutic effect was seen, mostly due to abolishment or marked improvement of severe, treatment resistant spasticity (n = 12). In two cases the effect could not be determined, two patients did not benefit. The median duration of treatment was 181 days (range 23-1429 days). Dosages to obtain a therapeutic effect varied from 0.08 to 1.0 mg/kg/d with a median of 0.33 mg/kg/d in patients with a documented therapeutic effect. When administered as an escalating dosage scheme, side effects were rare and only consisted in vomiting and restlessness (one patient each). No serious and enduring side effects occurred even in young children and/or over a longer period of time. CONCLUSIONS: In the majority of pediatric palliative patients the treatment with dronabinol showed promising effects in treatment resistant spasticity.


Assuntos
Dronabinol/uso terapêutico , Espasticidade Muscular/tratamento farmacológico , Cuidados Paliativos/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Relação Dose-Resposta a Droga , Dronabinol/administração & dosagem , Dronabinol/efeitos adversos , Resistência a Medicamentos , Feminino , Humanos , Lactente , Masculino , Espasticidade Muscular/etiologia , Tono Muscular/efeitos dos fármacos , Doenças Neurodegenerativas/complicações , Doenças Neurodegenerativas/tratamento farmacológico , Estudos Retrospectivos , Assistência Terminal , Resultado do Tratamento , Adulto Jovem
8.
Eur J Paediatr Neurol ; 20(2): 261-269, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26753538

RESUMO

INTRODUCTION: Brain tumors have the highest disease-related mortality rate of all pediatric cancers. The goal of this study was to determine whether all children with incurable brain tumors cared for by a pediatric palliative care team in a home setting suffer from the same symptoms towards the end of their lives or whether there are differences between the tumor localizations with implications for palliative care. PATIENTS AND METHODS: This study was conducted as a retrospective, single center chart review including all patients treated between January 1st 2000 and December 31st 2013. RESULTS: 70 children, adolescents and young adults were included in the analysis. Symptom burden was high with a mean number of symptoms of 7.2 per patient. 74% of the symptoms already existed one week before death. Within the last week of life, impaired consciousness (75.7%) most often occurred. Furthermore, symptoms considerably depended on tumor localization. Patients with supratentorial tumors presented more frequently with seizures (p < 0.05), coma (p < 0.01), nausea and emesis (p < 0.01). Ataxia (p < 0.001) occurred most frequently in infratentorial tumors and speech disturbances (p < 0.05), cranial nerve paralysis (p < 0.001), and tetraparesis (p < 0.001) in brain stem tumors. 84.3% of the patients needed analgesics, only 64.4% WHO class III analgesics. Anticonvulsants were given more often in supratentorial tumors (p < 0.01). CONCLUSIONS: Caring for a dying child suffering from a brain tumor needs increased awareness of the neurological deterioration. The symptom pattern strongly depends on the tumor localization and significantly differs between supratentorial, infratentorial and brain stem tumors.


Assuntos
Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/terapia , Serviços de Assistência Domiciliar , Cuidados Paliativos/métodos , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Adulto Jovem
9.
Eur J Pediatr ; 175(3): 321-7, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26411975

RESUMO

UNLABELLED: The aim of this study was to determine the distinct issues neonates/infants with life-limiting conditions and their families face during palliative home care and to enable physicians/caregivers to carefully address their needs. Data on home-based palliative care of all neonates and infants, who were being taken care of by our paediatric palliative care team between 2007 and 2014, was analysed. A total of 31 patients (pts) were analysed. The majority of patients (n = 17) were diagnosed with congenital malformations or chromosomal abnormalities. Twenty pts died, five of them in hospital. A high percentage of pts presented with swallowing incoordination (83.9%) and was fed either by nasogastric tube or percutaneous endoscopic gastrostomy. Of the pts, 71.0% were treated with analgesics, 45.2% were oxygen dependent, and 9.7% required mechanical ventilation. Highest mortality was seen in pts with perinatal complications (75%). In four (12.9%) pts, palliative home care could come to an end as their conditions substantially improved. CONCLUSIONS: Palliative treatment of neonates/very young infants with terminal conditions at home seems to be similar to that of older children and feasible in children even with unstable conditions. The spectrum of diagnoses, signs and symptoms varies from older children with swallowing incoordination and artificial nutrition being of particular importance.


Assuntos
Serviços de Assistência Domiciliar/estatística & dados numéricos , Cuidados Paliativos/estatística & dados numéricos , Assistência Terminal/estatística & dados numéricos , Cuidadores , Feminino , Alemanha , Hospitais Pediátricos , Humanos , Lactente , Mortalidade Infantil , Recém-Nascido , Masculino , Conforto do Paciente/estatística & dados numéricos
10.
Neuro Oncol ; 13(6): 680-9, 2011 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-21636712

RESUMO

Pediatric high-grade gliomas (HGGs) of the thalamic region account for up to 13% of pediatric HGGs and usually result in only anecdotal long-term survival. Because very little is known about these tumors, we aimed to further characterize them. In our series of 99 pediatric thalamic HGGs, there were no significant differences in survival between patients with tumors affecting the thalamus alone (including bithalamic lesions) and patients with tumors affecting the thalamus plus adjacent structures. Tumor resection (event-free survival/overall survival) and an early treatment response to radiotherapy/chemotherapy (event-free survival) had independent prognostic significance, as shown by Kaplan-Meier and multivariate Cox regression analyses. When we compared clinical characteristics and outcomes of pediatric thalamic HGG with those of pediatric (nonthalamic) supratentorial (n = 177) as well as pediatric pontine HGG (including diffuse intrinsic pontine gliomas; n = 234), we found that thalamic HGG shared more similarities with pontine than with supratentorial HGG, but overall, it appeared to represent a clinically distinct subgroup of pediatric HGG. The varying extent of tumor resection in the different tumor localizations may play some role in the observed clinical differences, as shown by multivariate Cox regression analyses, but the tumor site itself was also identified as an independent prognostic parameter. Thus, an additional location-specific effect on survival and/or tumor biology, despite different neurosurgical accessibility, has to be considered. Therefore, future investigations should try to further characterize the obviously site-specific heterogeneity of pediatric HGG on a molecular genetic basis.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/classificação , Neoplasias Encefálicas/patologia , Glioma/classificação , Glioma/secundário , Tálamo/patologia , Adolescente , Neoplasias Encefálicas/terapia , Criança , Pré-Escolar , Terapia Combinada , Feminino , Seguimentos , Glioma/terapia , Humanos , Lactente , Recém-Nascido , Masculino , Estadiamento de Neoplasias , Radioterapia , Taxa de Sobrevida , Resultado do Tratamento
11.
J Neurooncol ; 92(2): 157-63, 2009 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19043777

RESUMO

PURPOSE: Anaplastic gangliogliomas (AGG) are gangliogliomas with areas of pronounced hypercellularity, vascular proliferation, necrosis, and many mitotic figures. As very few pediatric patients have been studied, we analyzed the cases registered in the HIT-GBM database. PATIENTS AND METHODS: Patient data were obtained from the German HIT-GBM database. Inclusion criteria were diagnosis of AGG proven by a central neuropathological review and patient age 0 to 17 years. Eight patients (five male and three female) were identified. RESULTS: Patients' median age was 10 years. The median history of disease was 9 months (range, 1.0-43.0 months). Initial symptoms included signs of raised intracranial pressure, seizures, and, in the case of spinal cord tumor, bladder dysfunction. In five cases, AGGs were localized supratentorially with three patients having multiple lobes involved. The tumors affected the frontal (n = 3 cases), parietal (n = 2), temporal (n = 2), and occipital lobes (n = 1), as well as the brainstem (n = 1) and the spinal cord (n = 2). Gross total tumor resection was achieved in six patients. The estimated 5-year overall survival rate +/- standard error was 88 +/- 12%, and the event-free survival rate was 63 +/- 17%. While gender and tumor location did not affect survival rates, gross total tumor resection provided a better overall survival than non-total resection. CONCLUSION: The prognosis of pediatric patients with AGG is good, especially for those who undergo gross total tumor resection.


Assuntos
Neoplasias Encefálicas/patologia , Ganglioglioma/patologia , Adolescente , Neoplasias Encefálicas/fisiopatologia , Neoplasias Encefálicas/cirurgia , Criança , Pré-Escolar , Feminino , Ganglioglioma/fisiopatologia , Ganglioglioma/cirurgia , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Prognóstico
12.
Eur J Pediatr ; 167(12): 1449-52, 2008 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-18604554

RESUMO

Splenic infarction is a common cause of left upper quadrant pain and must be suspected in patients with hematologic or thromboembolic conditions and signs of localized or systemic inflammation. Although several mechanisms have been proposed for splenic infarction in patients with various hematologic disorders, hereditary spherocytosis (HS) is usually not associated with an increased risk for thromboembolic events. We report a 13-year-old male with HS who was referred to our hospital with a 4-day history of fever and left upper quadrant pain. Ultrasound scans and magnetic resonance imaging showed lesions suggestive of splenic infarction. Initially, antibiotic treatment was started because secondary infection was suspected. However, 1 week after admission the patient developed typical clinical signs of acute infectious mononucleosis. Further laboratory work up confirmed the diagnosis of acute Epstein-Barr virus infection and additionally revealed protein C deficiency. This association has not been reported previously and may have contributed to the development of splenic infarction. Since infectious mononucleosis is a common cause for clinical consultations in adolescence, physicians caring for children with hematologic disorders should be particularly aware of those possible complications.


Assuntos
Mononucleose Infecciosa/complicações , Deficiência de Proteína C/complicações , Esferocitose Hereditária/complicações , Infarto do Baço/etiologia , Doença Aguda , Adolescente , Humanos , Mononucleose Infecciosa/diagnóstico , Masculino , Deficiência de Proteína C/diagnóstico , Esferocitose Hereditária/diagnóstico , Infarto do Baço/diagnóstico , Infarto do Baço/virologia , Esplenomegalia
13.
Eur J Cancer ; 44(13): 1861-5, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18662870

RESUMO

BACKGROUND: Infections are significant causes of morbidity and mortality among immunocompromised patients, but little is known about the adherence by the paediatric cancer patients to preventive anti-infective interventions. METHODS: A voluntary and anonymised questionnaire was distributed to all patients, completing intensive anticancer therapy. Compliance was analysed by using a panel of eight commonly recommended preventive interventions and semi-quantitative scoring of adherence by the patient and/or its caretaker. Satisfaction with information and belief in the efficacy of the interventions were similarly assessed. Relationships of these factors to compliance were explored by using an overall compliance score and non-parametric correlation and/or ANOVA and logistic regression, respectively. RESULTS: In 216 children and adolescents (mean age: 8 years; 94 girls) included in the study, compliance rates were the highest for food restriction (89.3%), the use of topic antimycotics (88.2%) and trimethoprim/sulfamethoxazole (86.6%), and the lowest for the use of face masks (68.8%), antiseptic mouth rinses (67.1%), non-absorbable antibiotic agents (66.5%) and restrictions in social contacts (65.5%). The most frequent reasons for drug non-compliance were forgetfulness and patient refusal. Compliance correlated with haematological malignancy, younger age and belief in its efficacy, but not with the perceived degree of information, burden of interventions and overall satisfaction with quality of information and medical care. CONCLUSION: Compliance to recommended anti-infective prophylactic interventions was variable and correlated with haematological malignancy, younger age and belief in efficacy.


Assuntos
Anti-Infecciosos/uso terapêutico , Controle de Infecções/métodos , Neoplasias/tratamento farmacológico , Cooperação do Paciente , Adolescente , Adulto , Análise de Variância , Anti-Infecciosos Locais/administração & dosagem , Antifúngicos/uso terapêutico , Criança , Pré-Escolar , Efeitos Psicossociais da Doença , Feminino , Humanos , Lactente , Masculino , Satisfação do Paciente , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Adulto Jovem
14.
J Pediatr ; 153(1): 127-32, 2008 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-18571550

RESUMO

OBJECTIVE: To explore the frequency and course of neurodegenerative central nervous system (CNS) disease in Langerhans cell histiocytosis (ND-LCH). STUDY DESIGN: We studied 83 patients with LCH in whom magnetic resonance imaging (MRI) of the brain was performed at least twice for various clinical indications. We defined radiologic ND-LCH as an MRI pattern comprising bilateral symmetric lesions in the dentate nucleus of the cerebellum or basal ganglia. RESULTS: Forty-seven of 83 patients (57%) had radiologic ND-LCH, at a median of 34 months (range 0-16 years) from the diagnosis of LCH. The MRI findings deteriorated in 31/47 (66%) patients over a median of 3 years (range 2 months to 12 years 6 months) and did not reverse in any patient. In 12 patients with radiologic ND-LCH (25%), clinical ND-LCH with overt symptoms were found 3 to 15 years (median 6 years) after initial LCH diagnosis. These symptoms included intention tremor, cerebellar ataxia, dysarthria, dysdiadochokinesis, concentration deficits, psychomotor retardation, severe headaches, and psychosis. CONCLUSION: We conclude that radiologic ND-LCH is serious, not uncommon in patients studied by MRI, irreversible, and may be followed by severe clinical ND-LCH many years after the initial diagnosis of LCH.


Assuntos
Histiocitose de Células de Langerhans/diagnóstico , Histiocitose de Células de Langerhans/patologia , Doenças Neurodegenerativas/diagnóstico , Doenças Neurodegenerativas/patologia , Adolescente , Gânglios da Base/patologia , Núcleos Cerebelares/metabolismo , Cerebelo/metabolismo , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Imagem por Ressonância Magnética/métodos , Masculino , Fatores de Tempo
15.
J Neurooncol ; 86(2): 175-81, 2008 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-17641821

RESUMO

BACKGROUND: The combination of topoisomerase I and II chemotherapeutic agents has shown promising preclinical synergistic effects in the treatment of high-grade malignant brain tumors such as high-grade gliomas and choroid plexus carcinomas. To confirm the effectiveness of this treatment combination and determine its possible toxicity, we conducted a retrospective review of the charts of children who received the therapy. METHODS: Patients with relapsed malignant brain tumors who were given an individualized treatment of pegylated (PEG)-liposomal doxorubicin and topotecan were included in our study. PEG-liposomal doxorubicin was given intravenously at a dosage of 30-40 mg/m(2) over 4 h once every 4 weeks. Additionally, an intravenous formulation of topotecan was given orally twice daily and was increased on an individual basis from a starting dosage of 0.3 mg/m(2) per application to a total daily dosage of 0.6 mg/m(2). RESULTS: Eight patients were included. The main toxicity (NCI-CTC) after three cycles of the combination therapy was grade IV hematotoxicity (n = 3); grade III hematotoxicity (n = 2), grade III stomatitis (n = 1), grade III infection (n = 2), grade III diarrhea (n = 1); and grade II dermatitis (n = 1). In four patients, stable disease was achieved for 9, 23, more than 24, and more than 48 weeks, respectively. CONCLUSION: The schedule of PEG-liposomal doxorubicin with 30-40 mg/m(2) every 4 weeks in combination with oral topotecan resulted in tumor response, but the toxicity was high. An individualized increasing dose of PEG-liposomal doxorubicin 10-20 mg/m(2) every two weeks is now recommended.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Encefálicas/tratamento farmacológico , Glioblastoma/tratamento farmacológico , Recidiva Local de Neoplasia/tratamento farmacológico , Administração Oral , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias Encefálicas/patologia , Carcinoma/tratamento farmacológico , Carcinoma/patologia , Criança , Pré-Escolar , Neoplasias do Plexo Corióideo/tratamento farmacológico , Neoplasias do Plexo Corióideo/patologia , Intervalo Livre de Doença , Relação Dose-Resposta a Droga , Doxorrubicina/administração & dosagem , Doxorrubicina/análogos & derivados , Esquema de Medicação , Erupção por Droga , Glioblastoma/patologia , Doenças Hematológicas/induzido quimicamente , Humanos , Polietilenoglicóis/administração & dosagem , Recidiva , Estudos Retrospectivos , Estomatite/induzido quimicamente , Topotecan/administração & dosagem , Resultado do Tratamento
16.
J Neurooncol ; 79(2): 197-201, 2006 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-16598421

RESUMO

The association of weight loss and pediatric brain tumors that affect the diencephalon or brain stem with weight loss is a recognized, but not fully understood phenomenon. Tumors located in the hypothalamic region may induce the diencephalic syndrome (DS), which is characterized by profound emaciation with almost complete loss of subcutaneous fatty tissue. Tumors that compress or infiltrate the brain stem rarely cause both psychological disturbance and emaciation. The clinical presentation may be different, depending on the location of the lesion and age of the patient. In this report we present an unusual case of severe emaciation in a 4(9)/(12)-year-old girl with a juvenile pilocytic astrocytoma of the hypothalamic region and brain stem with neuroaxis dissemination. This case illustrates the importance of considering intracranial mass-lesions in the differential diagnosis of weight loss, psychological disturbance and atypical eating disorder. We discuss the importance of tumor multifocality and the role of patient age in the clinical presentation with reference to the literature.


Assuntos
Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Emaciação/etiologia , Transtornos da Alimentação e da Ingestão de Alimentos/etiologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Astrocitoma/complicações , Astrocitoma/tratamento farmacológico , Neoplasias Encefálicas/complicações , Neoplasias Encefálicas/tratamento farmacológico , Tronco Encefálico/patologia , Pré-Escolar , Diagnóstico Diferencial , Emaciação/patologia , Emaciação/cirurgia , Transtornos da Alimentação e da Ingestão de Alimentos/patologia , Transtornos da Alimentação e da Ingestão de Alimentos/cirurgia , Transtornos de Alimentação na Infância/diagnóstico , Feminino , Humanos , Hipotálamo/patologia , Neoplasias da Medula Espinal/complicações , Neoplasias da Medula Espinal/secundário , Resultado do Tratamento
17.
Eur J Pain ; 9(4): 395-406, 2005 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-15979020

RESUMO

There is a lack of valid epidemiological data on malignancy-associated pain in modern pediatric oncology. Pediatric oncology patients (self-assessment) and their parents from 28 hospitals were questioned using age-adapted, structured interviews and validated pain assessment tools. Pain intensity was measured by the NRS and Bieri faces scale. We conducted 363 interviews with patients and their parents, and 46 with the parents alone (if patients <2.5 years). Pain was reported at the time of the interview or within the last 24 h, 7 d, or 4 weeks in 15%, 28%, 50% and 58% of cases, respectively. The proportion of patients suffering severe to maximal pain (NRS>3; Bieri>2) increased significantly (p=0.001, chi2 test). The median pain intensity for the most severe pain episode within the last 4 weeks was 6.7 (NRS 0-10). Adverse effects of anti-tumor therapy were the most frequent cause of pain. Multivariate analyses depicted general physical condition either "severely reduced" (ASA status 3) (OR 4.0, 95% CI 1.1-14.7, p=0.037) or "moderately reduced" (ASA status 2) (OR 1.8, 95% CI 1.1-2.9, p=0.018), "in-patient status" (OR 1.8, 95% CI 1.2-2.9, p=0.010), and "co-morbidity present" (OR 3.5, 95% CI 1.1-10.7, p=0.030) as risk factors for severe to maximal pain. General anesthesia was the only factor significantly (OR 0.14, 95% CI 0.05-0.39, p<0.01) associated with a reduction in the proportion of patients suffering severe to maximal pain during bone marrow aspiration. Our data emphasize both the importance of in-house acute pain control and the need for general anesthesia during painful procedures in pediatric oncology.


Assuntos
Antineoplásicos/efeitos adversos , Biópsia por Agulha/efeitos adversos , Neoplasias/complicações , Dor/epidemiologia , Radioterapia/efeitos adversos , Punção Espinal/efeitos adversos , Adolescente , Analgésicos/uso terapêutico , Anestesia Geral/normas , Medula Óssea/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Análise Multivariada , Neoplasias/terapia , Dor/etiologia , Dor/psicologia , Medição da Dor , Relações Pais-Filho , Satisfação do Paciente , Fatores de Risco , Punção Espinal/psicologia , Inquéritos e Questionários
18.
Pediatr Hematol Oncol ; 20(2): 167-72, 2003 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-12554529

RESUMO

One patient with a chromosome 22q11.2 deletion and Evans syndrome is reported in this paper. Microdeletions of 22q11.2 are the main etiology for DiGeorge syndrome, a disorder characterized by heart defects, immune deficiencies due to aplasia or hypoplasia of the thymus, and hypocalcemia. Evans syndrome refers to a hematological autoimmune disorder with autoimmune hemolytic anemia accompanied by immune thrombocytopenia. A wide range of autoimmune disorders have been described in DiGeorge syndrome and velocardiofacial syndrome, including one prior report of autoimmune hemolytic anemia and immune thrombocytopenia. The patient reported herein strengthens the association between the 22q11.2 deletion spectrum and Evans syndrome.


Assuntos
Anemia Hemolítica Autoimune/genética , Doenças Autoimunes/genética , Deleção Cromossômica , Cromossomos Humanos Par 22/ultraestrutura , Dupla Via de Saída do Ventrículo Direito/genética , Trombocitopenia/genética , Anemia Hemolítica Autoimune/tratamento farmacológico , Atrofia , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/imunologia , Encéfalo/patologia , Síndrome de DiGeorge/diagnóstico , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Lactente , Infecção/etiologia , Deficiência Intelectual/genética , Deficiência Intelectual/patologia , Prednisona/efeitos adversos , Prednisona/uso terapêutico , Recidiva , Síndrome , Trombocitopenia/tratamento farmacológico , Trombocitopenia/imunologia , Insuficiência Velofaríngea/genética
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