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Biomedical subjects

Joanne M Hilden

Publications and source records attributed to Joanne M Hilden.

15 recordsLinked to original sources
PubMed2007

Birth characteristics, maternal reproductive history, and the risk of infant leukemia: a report from the Children's Oncology Group.

Leukemias with MLL gene rearrangements predominate in infants (<1 year of age), but not in older children, and may have a distinct etiology. High birth weight, higher birth order, and prior fetal loss have, with varying consistency, been associated with infant leukemia, but no studies have reported results with respect to MLL status. Here, we report for the first time such an analysis. During 1999 to 2003, mothers of 240 incident cases (113 MLL(+), 80 MLL(-), and 47 indeterminate) and 255 random digit dialed controls completed a telephone interview. Odds ratios and 95% confidence intervals for quartile of birth weight, birth order, gestational age, maternal age at delivery, prior fetal loss, pre-pregnancy body mass index, and weight gain during pregnancy were obtained using unconditional logistic regression; P for linear trend was obtained by modeling continuous variables. There was a borderline significant linear trend of increasing birth weight with MLL(+) (P = 0.06), but not MLL(-) (P = 0.93), infant leukemia. Increasing birth order showed a significant inverse linear trend, independent of birth weight, with MLL(+) (P = 0.01), but not MLL(-) (P = 0.18), infant leukemia. Other variables of interest were not notably associated with infant leukemia regardless of MLL status. This investigation further supports the contention that molecularly defined subtypes of infant leukemia have separate etiologies.

Adult↗
PubMed2006

Analysis of prognostic factors of acute lymphoblastic leukemia in infants: report on CCG 1953 from the Children's Oncology Group.

Infant acute lymphoblastic leukemia (ALL) has a poor therapeutic outcome despite attempts to treat it based on prognostic factor-guided therapy. This is the first cooperative group trial characterizing all infants at the molecular level for MLL/11q23 rearrangement. All infants enrolled on Children's Cancer Group (CCG) 1953 were tested for MLL rearrangement by Southern blot and the 11q23 translocation partner was identified (4;11, 9;11, 11;19, or "other") by reverse-transcriptase polymerase chain reaction (PCR). One hundred fifteen infants were enrolled; overall event-free survival (EFS) was 41.7% (SD = 9.2%) and overall survival (OS) was 44.8% at 5 years. Five-year EFS for MLL-rearranged cases was 33.6% and for MLL-nonrearranged cases was 60.3%. The difference in EFS between the 3 major MLL rearrangements did not reach statistical significance. Multivariate Cox regression analyses showed a rank order of significance for negative impact on prognosis of CD10 negativity, age younger than 6 months, and MLL rearrangement, in that order. Toxicity was the most frequent cause of death. Relapse as a first event in CCG 1953 was later (median, 295 days) compared with CCG 1883 historic control (median, 207 days). MLL/11q23 rearrangement, CD10 expression, and age are important prognostic factors in infant ALL, but molecular 11q23 translocation partners do not predict outcome.

Age Factors↗
PubMed2006

Complementary and alternative medicine in pediatric oncology: availability and institutional policies in Canada--a report from the Children's Oncology Group.

Complementary and alternative medicine (CAM) is used frequently by pediatric oncology patients. A survey exploring the institutional practices and policies surrounding CAM use in pediatric oncology patients was completed by 17 pediatric hematology/oncology centers in Canada. We found that CAM was offered in only 18% of the institutions, but 94% of the communities. Only 6% of oncology divisions made direct referrals to community CAM providers, and only 20% of the centers had policies regarding use of CAM therapies for their patients. Despite published widespread use of CAM therapies, our study demonstrates that institutional CAM resources and policies on CAM are present in much lower proportions.

Canada↗
PubMed2006

Program interventions for children at the end of life and their siblings.

An analogy may be drawn between readying a family for the birthof a child and readying a family for the death of a child. Both ex-periences bring about an intense fusion of the emotional, physical,and spiritual realms for those bearing witness. Preparation, com-munication, and collaboration are essential to provide optimalsupport for the children at the end of life, the parents, and thebrothers and sisters.

Bereavement↗
PubMed2006

From cure to palliation: managing the transition.

When a child is diagnosed with a life-threatening or life-limiting condition, their physical and affective world is immediately changed, regardless of the outcome of treatment. Pediatric care providers are in a unique position to offer hope to children and families even when cure is not possible. This hope focuses not only on cure, but incorporates the understanding that quality of life is not commensurate with length of life and that compassionate care can be provided in all stages of treatment.

Attitude to Death↗
PubMed2005

Parent and physician perspectives on quality of care at the end of life in children with cancer.

PURPOSE: To ascertain parents' and physicians' assessments of quality of end-of-life care for children with cancer and to determine factors associated with high-quality care as perceived by parents and physicians. METHODS: A survey was conducted between 1997 and 2001 of 144 parents of children who received treatment at the Dana-Farber Cancer Institute and Children's Hospital (Boston, MA) or Children's Hospitals and Clinics of St Paul and Minneapolis, MN, between 1990 and 1999 (65% of those located and eligible) and 52 pediatric oncologists. RESULTS: In multivariable models, higher parent ratings of physician care were associated with physicians giving clear information about what to expect in the end-of-life period (odds ratio [OR] = 19.90, P = .02), communicating with care and sensitivity (OR = 7.67, P < .01), communicating directly with the child when appropriate (OR = 11.18, P < .01), and preparing the parent for circumstances surrounding the child's death (OR = 4.84, P = .03). Parent reports of the child's pain and suffering were not significant correlates of parental ratings of care (P = .93 and .35, respectively). Oncologists' ratings of care were inversely associated with the parent's report of the child's experience of pain (OR = 0.15, P = .01) and more than 10 hospital days in the last month of life (OR = 0.24, P < .01). Parent-rated communication factors were not correlates of oncologist-rated care. No association was found between parent and physician care ratings (P = .88). CONCLUSION: For parents of children who die of cancer, doctor-patient communication is the principal determinant of high-quality physician care. In contrast, physicians' care ratings depend on biomedical rather than relational aspects of care.

Adolescent↗
PubMed2005

Issues and challenges in palliative care for children with cancer.

Although the majority of children with cancer are cured of their illness, the children who die from their disease or complications require special care at the end of life. We present special issues and challenges unique to pediatric palliative care and suggest ways in which we can face these issues and address the challenges. The care must be family centered and balance the needs of the health-care system, the child, and the family. The way in which the care is delivered, the services provided, and the place in which that care is given are not carved into a simple protocol. Quality of life is an important concept that is often overlooked. Educational initiatives for patients, families, health-care providers, and third-party payers are essential. Reimbursement for palliative care services presents a large barrier to provision of appropriate services to all children and families in need. Hypothesis-driven research must be developed to help us learn more about how best to deliver end-of-life care to children and their families.

Adolescent↗
PubMed2005

Maternal diet and infant leukemia: the DNA topoisomerase II inhibitor hypothesis: a report from the children's oncology group.

BACKGROUND: The MLL 11q23 translocation arises in utero and is present in 75% of infant leukemias. That MLL+ acute myeloid leukemia (AML) can arise following chemotherapy with DNA topoisomerase II (DNAt2) inhibitors suggests that these substances, which also occur naturally in foods, may contribute toward infant leukemia. We hypothesized that maternal consumption of dietary DNAt2 inhibitors during pregnancy would increase the risk of infant leukemia, particularly AML(MLL+). METHODS: This Children's Oncology Group case-control study consisted of 240 incident cases of infant acute leukemia [AML and acute lymphoblastic leukemia (ALL)] diagnosed during 1996 to 2002 and 255 random digit dialed controls. Maternal diet during pregnancy was determined through a food frequency questionnaire. An index of specific foods identified a priori to contain DNAt2 inhibitors as well as vegetables and fruits were created and analyzed using unconditional logistic regression. RESULTS: There was little evidence of an association between the specific DNAt2 index and leukemia overall and by subtype. An exception was AML(MLL+); odds ratios (95% confidence intervals) comparing the second to fourth quartiles to the first were 1.9 (0.5-7.0), 2.1 (0.6-7.7), and 3.2 (0.9-11.9), respectively (P for trend = 0.10). For the vegetable and fruit index, there were significant or near-significant inverse linear trends for all leukemias combined, ALL(MLL+), and AML(MLL-). CONCLUSION: Overall, maternal consumption of fresh vegetables and fruits during pregnancy was associated with a decreased risk of infant leukemia, particularly MLL+. However, for AML(MLL+) cases, maternal consumption of specific DNAt2 inhibitors seemed to increase risk. Although based on small numbers, these data provide some support for distinct etiologic pathways in infant leukemia.

Acute Disease↗
PubMed2004

Central nervous system atypical teratoid/rhabdoid tumor: results of therapy in children enrolled in a registry.

PURPOSE: Atypical teratoid/rhabdoid tumor (AT/RT) of the CNS is an extremely rare and aggressive tumor of early childhood. The poor outcome with conventional infant brain tumor therapy has resulted in a lack of clear treatment guidelines. A registry has been established to create an outcomes database and to facilitate biology studies for this tumor. MATERIALS AND METHODS: A standardized data sheet was provided to treating physicians listing the reports that were to be sent to the registry for abstraction. Follow-up information was sought twice yearly. RESULTS: Information was complete for 42 patients. Median age at diagnosis was 24 months. Nine patients (21%) had disseminated disease at diagnosis. Sixteen tumors were infratentorial; 26 were supratentorial. Twenty patients (48%) received a primary complete resection. Primary therapy included chemotherapy in all patients, radiotherapy in 13 patients (31%), stem-cell rescue in 13 patients (31%), and intrathecal chemotherapy in 16 patients (38%). Recurrent or progressive disease was reported in nine and 19 patients, respectively. Twenty-seven patients (64%) are dead of disease (3 to 62 months from diagnosis) and one patient died of toxicity. Fourteen patients (33%) show no evidence of disease (9.5 to 96 months from diagnosis). The median survival is 16.75 months and the median event-free survival is 10 months. CONCLUSION: Aggressive therapy has prolonged the natural history in a subset of children. Prospective multi-institutional and national clinical trials designed specifically for AT/RT are needed. Enrollment onto the AT/RT registry should be continued.

Central Nervous System Neoplasms↗
PubMed2004

Issues and challenges in palliative care for children with cancer.

Although the majority of children with cancer are cured of their illness, the children who die from their disease or complications require special care at the end of life. We present special issues and challenges unique to pediatric palliative care and suggest ways in which we can face these issues and address the challenges. The care must be family centered and balance the needs of the health-care system, the child, and the family. The way in which the care is delivered, the services provided, and the place in which that care is given are not carved into a simple protocol. Quality of life is an important concept that is often overlooked. Educational initiatives for patients, families, health-care providers, and third-party payers are essential. Reimbursement for palliative care services presents a large barrier to provision of appropriate services to all children and families in need. Hypothesis-driven research must be developed to help us learn more about how best to deliver end-of-life care to children and their families.

Child↗
PubMed2004

Brain tumors in children.

Pediatric brain tumors are a challenging group of very divergent diseases. Considerable controversy exists concerning the accurate diagnosis and treatment of these patients. Aggressive therapy is often needed to cure aggressive and potentially lethal disease, yet late effects, especially injury to the developing brain, remain a profound problem. The discipline of pediatric neuro-oncology remains one of the most productive areas for continued clinical and basic research because of these and other challenges.

Biomedical Research↗
PubMed2003

Tell the children.

Explore the source record for details and available documents.

Attitude to Death↗
PubMed2002

A randomized comparison of native Escherichia coli asparaginase and polyethylene glycol conjugated asparaginase for treatment of children with newly diagnosed standard-risk acute lymphoblastic leukemia: a Children's Cancer Group study.

For this study, 118 children with standard-risk acute lymphoblastic leukemia (ALL) were given randomized assignments to receive native or pegylated Escherichia coli asparaginase as part of induction and 2 delayed intensification phases. Patients treated with pegaspargase had more rapid clearance of lymphoblasts from day 7 and day 14 bone marrow aspirates and more prolonged asparaginase activity than those treated with native asparaginase. In the first delayed intensification phase, 26% of native asparaginase patients had high-titer antibodies, whereas 2% of pegaspargase patients had those levels. High-titer antibodies were associated with low asparaginase activity in the native arm, but not in the pegaspargase arm. Adverse events, infections, and hospitalization were similar between arms. Event-free survival at 3 years was 82%. A population pharmacodynamic model using the nonlinear mixed effects model (NONMEM) program was developed that closely fit the measured enzyme activity and asparagine concentrations. Half-lives of asparaginase were 5.5 days and 26 hours for pegaspargase and native asparaginase, respectively. There was correlation between asparaginase enzymatic activity and depletion of asparagine or glutamine in serum. In cerebrospinal fluid asparagine, depletion was similar with both enzyme preparations. Intensive pegaspargase for newly diagnosed ALL should be tested further in a larger population.

Amino Acids↗