ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The biological and clinical characteristics of perinatal leukemia differ significantly from those of leukemia in older children, and the prognosis is generally bleak. Once complete remission is achieved, neonates with acute myelocytic leukemia (AML) fare much better than those with acute lymphocytic leukemia (ALL). The results of this study suggest that age, sex, type of leukemia, and cytogenetic findings have a strong influence on outcome. Neonates, particularly females, with pre-B ALL have a much worse prognosis than neonates and older children with this disease. Transient leukemia in the Down syndrome neonate is associated with significant morbidity; close follow-up is recommended for at least the first 3 years of life because of the potential of developing acute leukemia, particularly AMKL (M7). The purpose of this review is to focus on the fetus and neonate in an attempt to determine the various ways leukemia differs clinically and morphologically from the disease occurring in older infants and children and to demonstrate that certain types of leukemia have a poor prognosis compared with those occurring in older children.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

PurposeTo analyze the genetic and epigenetic alterations affecting the RB1, TP53, p16INK4, and p21WAF1 tumor suppressor genes, loss of heterozygosity (LOH) at 3q and 18q, and the clinical variables of a series of Spanish children with osteosarcoma. These genetic changes were tested for an association with prognosis.MethodsPeripheral blood samples and clinical data were available from 76 patients with osteosarcoma. Paired tissue was available from 41 of them. The mutation and methylation status of p16INK4, p21WAF1, TP53, and RB1 was screened as well as LOH at 3q and 18q.ResultsLoss of heterozygosity affecting RB1 (37.2%), TP53 (42.3%), and 18q (30.8%) and TP53 mutation (39%) were frequently encountered. TP53 mutation was associated with diagnosis at a later age. RB1 alteration was associated with reduced survival and event-free survival. The clinical variables associated with poor prognosis were the presence of metastasis at diagnosis (P= 0.035) or during treatment (P= 0.016) and the chondroblastic histologic subtype (P= 0.007); the response to induction chemotherapy (<90% necrosis) also tended to be related to poor prognosis (P= 0.08).ConclusionsRB1, TP53, and possibly other tumor suppressor genes located at 18q and other localizations are involved in pediatric osteosarcoma carcinogenesis, together with other genetic alterations not fully understood to date. Based on these results, the presence of an altered RB1 gene should be regarded as a poor prognostic factor for pediatric osteosarcoma.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

PurposeTo evaluate how parents viewed the authors' practice of audiotaping the initial communication of the leukemia diagnosis and of the complete program of care, including the prognosis.MethodsFrom January 1997 through December 1998, at the end of the formal communication interview, parents were asked to evaluate the audiotaping by filling out a questionnaire concerning the procedure.ResultsSixty-three parents of children with leukemia participated.ConclusionsThe authors' use of audiotapes was strongly supported by the parents as a supplementary intervention and has become a routine procedure.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

PurposeTo assess the activity and tolerability of temozolomide in children with progressive low-grade gliomas (LGGs).Patients and MethodsThe authors reviewed the records of 13 children (6 months to 19 years old) with progressive LGGs and magnetic resonance imaging evidence of unresectable tumors who were treated with temozolomide at the authors' institution since 1999.ResultsFour patients received a 5-day regimen of temozolomide (150 mg/m2per day) repeated every 28 days, and nine patients received a 42-day regimen (75 mg/m2per day) repeated every 56 days. Three patients demonstrated partial responses to temozolomide, with a median time to maximal response of 5 months (range 4–12 months), and one had a minor response at 9 months. Four patients developed progression while on temozolomide, with a median time to progression of 7 months (range 1–12 months). Five patients had disease stabilization. Among the five patients with prior chemotherapy and/or radiation therapy, temozolomide was associated with disease stabilization in three and tumor response in one. In the three patients with neurofibromatosis type 1, two patients experienced tumor responses and one disease stabilization. Thrombocytopenia, nausea, emesis, and fatigue were the most common toxicities. Four patients discontinued therapy because of the side effects.ConclusionsTemozolomide is active in children with LGGs. It is effective in previously treated patients and in patients with neurofibromatosis type 1. The 42-day regimen appears less toxic than the 5-day regimen. Any impact on survival for these patients remains to be demonstrated.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

PurposeTo compare results of low-dose tissue plasminogen activator (TPA) in children with arterial and venous thrombi relative to standard published dosing.MethodsSubjects consisted of all consecutive children with objectively confirmed thrombi for whom TPA thrombolysis was clinically ordered by the authors. Initial dosing used published standard dose (0.1–0.5 mg/kg per hour). With experience, a low-dose regimen (0.01–0.06 mg/kg per hour) was given in an attempt to derive a minimal effective dose.ResultsThirty-five children were treated with TPA. Either standard or low-dose infusions of TPA resulted in complete thrombolysis of 28 of 29 (97%) acute thrombi, while all 6 chronic thrombi had a partial response. In contrast to the recommended adult-derived dosages of 0.1 to 0.5 mg/kg per hour, the authors found that initial doses of less than 0.01 mg/kg per hour were effective in 12 of 17 patients with acute thrombosis. Neonates required 0.06 mg/kg per hour. Route of administration (local or systemic) did not affect efficacy. Major bleeding occurred in only one extremely preterm infant. Minor bleeding, primarily oozing at intravenous sites, occurred in 27% of children during TPA infusions. Prophylactic unfractionated or low-molecular-weight heparin was infused concomitant with TPA in 42% of the children and did not increase the risk of bleeding.ConclusionsTPA in very low doses appears to be safe and effective for thrombolysis of acute thromboses in most children, given appropriate patient selection.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

PurposeTo investigate the prevalence and clinical consequences of previous parvovirus B19 exposure in a large cohort of pediatric patients with sickle cell anemia (SCA).MethodsProspective serologic testing for previous parvovirus B19 exposure was performed in steady-state pediatric patients with SCA, either prior to starting hydroxyurea therapy or in preparation for transition to the adult service. A retrospective chart review was performed to ascertain whether patients had a documented history of a transient aplastic crisis.ResultsThe prevalence of serologic evidence of previous parvovirus infection increased with age. The overall prevalence in 102 children with SCA was 53%, ranging from 44% between 5 and 9 years of age to 71% between 17 and 21 years of age. Only 27% of patients had a previous clinically recognized transient aplastic crisis.ConclusionsBy the teenage years, most pediatric patients with SCA have serologic evidence of previous parvovirus B19 exposure. However, subclinical parvovirus infection appears to be common in children with SCA, since most patients have no documented previous transient aplastic crisis.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

PurposeTo prospectively identify all cases of bacteremia in children with sickle cell disease (SCD), establish time to positivity for various microorganisms, correlate clinical findings with microbiology data, and determine the antibiotic resistance pattern of the pneumococcal isolates.MethodsAll positive blood cultures from children with SCD followed at the Children's Hospital of Philadelphia from January 1993 through May 2001 were included. Isolates were classified as pathogen or contaminant. Demographic and clinical information was abstracted from the medical records. Time to positivity and antibiotic resistance data were generated in the microbiology laboratory.ResultsOne hundred forty-one positive blood culture bottles were obtained during distinct febrile episodes. Thirty-nine percent contained pathogens and 61% contained contaminants. The average time to positivity was 17.1 hours in the pathogen group and 29.5 hours in the contaminant group (P< 0.0001).Streptococcus pneumoniaewas the most common pathogen (42% of total), with a mean patient age of 3.5 years. Gram-negative rods were the second most common organism (28% of total), with a mean patient age of 8.1 years. Thirty-one percent of the pneumococcal isolates were resistant to penicillin. Thirty-five percent of the pneumococcal isolates grew from children with a focus of infection. Acute chest syndrome was noted in 26% of patients with a positive blood culture forS. pneumoniae.Sixty-seven percent ofSalmonellaisolates and 50% ofStaphylococcus aureusisolates grew from patients who developed osteomyelitis.ConclusionsThe average time to positivity for pathogens can be used in conjunction with other factors to determine the length of observation required for children with SCD who present with febrile illness. Chest radiographs should be obtained on children with SCD who are bacteremic withS. pneumoniae.Bone scans should be obtained on children with SCD who are bacteremic withSalmonellaorS. aureus.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

The authors report a unique translocation in a patient with M7 acute myeloid leukemia and review the literature. A 22-month-old girl without Down syndrome was diagnosed with acute myeloid leukemia, subtype M7 (AML-M7), and died with relapsed disease following bone marrow transplantation. Tumor cells were evaluated using cytogenetics (including spectral karyotyping), immunohistochemistry, and flow cytometry. The patient was found to have a previously unreported complex translocation as follows: 50,XX,der(1)t(1;5)(p36?.1;p15?.1),del(5)(p15?.1), +6,+der(6;7)(?;?),der(7)t(6;7)(?;p22)[2],der(9)t(6;9) (?;p21)t(9;14)(q34;q11.2-q13),+10,t(12;16)(p13;q24),−14[2], del(14)(q13)[2],+der(19)t(1;19)(?;p13.3),+22[cp 4]. AML-M7 in non-Down syndrome patients is a rare disease that requires improved prognostic markers.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID

摘要:

All-trans-retinoic acid (ATRA) has a dramatic antitumor effect in patients with acute promyelocytic leukemia (APL). It is hepatically metabolized by cytochrome P-450, and there are known toxicities associated with high levels of this drug. The effects of ATRA can be potentiated by inhibition of cytochrome P-450, which is known to occur with certain drugs. We report a case of a patient with ATRA toxicity thought to be secondary to interaction with fluconazole.

ISSN:1077-4114    

出版商:OVID    

年代:2003

数据来源: OVID