Biomedical subjects

Julie Blatt

Publications and source records attributed to Julie Blatt.

13 recordsLinked to original sources

PubMed2008

Response to rituximab in a child with neuroblastoma and opsoclonus-myoclonus.

Opsoclonus-myoclonus (OM) is a paraneoplastic syndrome of probable autoimmune origin. Despite current therapies aimed at decreasing autoantibody formation, OM is difficult to control and may impact long-term neurologic outcome. We present a case of a 19-month-old patient who initially presented with OM, neuroblastoma and a constitutional cytogenetic abnormality t(5;12)(q11.2;q15). The patient's OM was recalcitrant to conventional therapies, but showed significant improvement following treatment with rituximab.

Antibodies, Monoclonal↗

PubMed2007

Thymic carcinoma in a child with HIV infection.

HIV infection predisposes to cancer during childhood. In addition to the AIDS-defining non-Hodgkin lymphoma (NHL) and Kaposi sarcoma, a range of other lymphoid malignancies and solid tumors have been described. We report the first case of an HIV-positive child with thymic carcinoma in the setting of regressing thymic cysts. The tumor expressed CKIT but failed to respond to imatinab mesylate after a transient response to multiagent chemotherapy. This case extends the spectrum of pediatric malignancy in the setting of HIV and suggests that patients with presumed benign thymic cysts require ongoing surveillance.

Antineoplastic Agents↗

PubMed2006

Visceral larval migrans masquerading as metastatic disease in a toddler with Wilms tumor.

A 22-month-old girl with a renal mass had multiple small pulmonary nodules on CT at her initial presentation. After biopsy and neoadjuvant chemotherapy, a Wilms tumor was resected and the pulmonary nodules were shown to have regressed on CT. Follow-up imaging 4 months after initial diagnosis demonstrated multiple new liver lesions and new pulmonary nodules with peripheral eosinophilia. Lung biopsy revealed granuloma formation with prominent eosinophils. The serum antibody titers for Toxocara canis were elevated. This case illustrates that toxocariasis should be considered as a rare differential diagnostic possibility for multiple liver lesions and multifocal peripheral pulmonary opacities in young children with Wilms tumor.

Animals↗

PubMed2006

Hearing genes and cisplatin deafness: a pilot study.

OBJECTIVE: Cisplatin commonly is used to treat pediatric solid tumors. A major dose-limiting toxicity is sensorineural hearing loss. Which patients experience ototoxicity after treatment with cisplatin must reflect individual susceptibility, since it is not seen in all similarly treated patients. We hypothesized that mutations or polymorphisms in hearing genes are more common in patients who experience ototoxicity than in the general population. STUDY DESIGN: We completed retrospective mutation screening of GJB2 and SLC26A4 and screened for three mtDNA mutations in patients with a history of childhood cancer who developed severe hearing loss at cumulative cisplatin doses of less than 400 mg/M2. MATERIALS AND METHODS: Patients younger than 21 years of age who experienced severe hearing loss after cisplatin were identified using the Childhood Cancer Survivor Study database. Archival DNA from buccal washes of 11 patients was used for mutation screening and detection. RESULTS: With the exception of one patient (9.1%) who was a carrier for the 35delG mutation, no mutant alleles were found. Given the reported prevalence of the 35delG mutation in the general population of 2.5%, this result is not significant (P = .35). CONCLUSIONS: It is not likely that any of the five hearing genes we examined contribute to cisplatin ototoxicity. Further study may be warranted to look at other hearing genes as possible predictors of cisplatin ototoxicity.

Adolescent↗

PubMed2005

Positive association between congenital anomalies and risk of neuroblastoma.

BACKGROUND: Case reports and epidemiological studies have suggested a relationship between congenital anomalies and childhood cancer, but some potential associations remain inconsistent. In this study, we investigated the association between congenital anomalies and neuroblastoma. PROCEDURE: We used data of a case-control study on neuroblastoma conducted from 1992 to 1994, including 538 children aged 0-19 years with newly diagnosed, histologically confirmed neuroblastoma and 504 controls identified by telephone random-digit dialing and matched to cases on date of birth. Information on congenital anomalies and potential confounding factors was collected through maternal telephone interviews using a structured questionnaire. We estimated odds ratios (OR) and 95% confidence intervals (CI), adjusted for reference age at diagnosis, mother's educational level, mother's race, and household income at birth. RESULTS: An association between the maternal report of any congenital anomalies and neuroblastoma (OR = 2.58; CI = 1.57-4.25) was observed. Neuroblastoma risk increased with increasing number of anomalies per child (OR = 3.90, CI = 1.27-11.9 for two anomalies or more), and when we restricted analyses to major anomalies (OR = 7.53, CI = 2.23-25.5). Genitourinary anomalies (OR = 5.84, CI = 1.67-20.4) and cardiac anomalies (OR = 4.27, CI = 1.22-15.0) had an elevated, but imprecise neuroblastoma risk. CONCLUSIONS: Our findings support the hypothesis of an association between neuroblastoma and congenital, especially urogenital and cardiac, anomalies.

Adolescent↗

PubMed2005

Multifocal bone marrow involvement in cat-scratch disease.

We describe 2 children with prolonged fever of unknown origin and prominent skeletal pain who had multifocal bone disease caused by Bartonella infection. Initial radiologic studies, including plain films, radionuclide scintigraphy and computed tomography, yielded negative results. In both cases, magnetic resonance imaging revealed multiple enhancing bone marrow lesions consistent with clinical symptoms. Microbiologic diagnoses were established serologically.

Animals↗

PubMed2004

Development of risk-based guidelines for pediatric cancer survivors: the Children's Oncology Group Long-Term Follow-Up Guidelines from the Children's Oncology Group Late Effects Committee and Nursing Discipline.

The Children's Oncology Group Long-Term Follow-Up Guidelines for Survivors of Childhood, Adolescent, and Young Adult Cancers are risk-based, exposure-related clinical practice guidelines intended to promote earlier detection of and intervention for complications that may potentially arise as a result of treatment for pediatric malignancies. Developed through the collaborative efforts of the Children's Oncology Group Late Effects Committee, Nursing Discipline, and Patient Advocacy Committee, these guidelines represent a statement of consensus from a multidisciplinary panel of experts in the late effects of pediatric cancer treatment. The guidelines are both evidence-based (utilizing established associations between therapeutic exposures and late effects to identify high-risk categories) and grounded in the collective clinical experience of experts (matching the magnitude of risk with the intensity of screening recommendations). They are intended for use beginning 2 or more years following the completion of cancer therapy; however, they are not intended to provide guidance for follow-up of the survivor's primary disease. A complementary set of patient education materials ("Health Links") was developed to enhance follow-up care and broaden the application of the guidelines. The information provided in these guidelines is important for health care providers in the fields of pediatrics, oncology, internal medicine, family practice, and gynecology, as well as subspecialists in many fields. Implementation of these guidelines is intended to increase awareness of potential late effects and to standardize and enhance follow-up care provided to survivors of pediatric cancer throughout the lifespan. The Guidelines, and related Health Links, can be downloaded in their entirety at www.survivorshipguidelines.org.

Adolescent↗

PubMed2004

Maternal medication use and neuroblastoma in offspring.

The association between a mother's use of specific medications during pregnancy and lactation and neuroblastoma in her offspring was evaluated in a case-control study. Newly diagnosed cases of neuroblastoma (n=504) in the United States and Canada were identified between 1992 and 1994 at 139 hospitals affiliated with the Pediatric Oncology Group or the Children's Cancer Group clinical trial programs. One age-matched control was sampled from the community of each case by means of random digit dialing. Exposure information was ascertained retrospectively from mothers in a structured telephone interview. Odds ratios were estimated using conditional logistic regression, with adjustment for maternal sociodemographic factors. The results did not support an association between neuroblastoma and maternal exposure to diuretic agents, antiinfective agents, estrogens, progestins, sedatives, anticonvulsant drugs, or drugs that may form N-nitroso derivatives. Mothers of cases were more likely to report using medications containing opioid agonists while they were pregnant or nursing than were mothers of controls (odds ratio=2.4, 95% confidence interval: 1.3, 4.3). Specifically, more mothers of cases reported using medications containing codeine while pregnant or nursing than did mothers of controls (odds ratio=3.4, 95% confidence interval: 1.4, 8.4). This preliminary finding may be due to bias, confounding, or chance, and additional studies are needed for confirmation.

Adult↗

PubMed2003

Ovarian carcinoma in an adolescent with transgenerational exposure to diethylstilbestrol.

Small cell carcinoma of the ovary is rare in adolescence. The etiology of such tumors is unknown. Several reports suggest that transgenerational exposure to diethylstilbestrol (DES) may have deleterious effects on the genitourinary tract. The authors describe a 15-year-old girl with small cell carcinoma of the ovary whose maternal grandmother had been taking DES while she was pregnant with the patient's mother. This case, while anecdotal, suggests that a transgenerational history of DES exposure should be noted, and that granddaughters with persistent abdominal pain even during childhood may need evaluation for genital tract abnormalities.

Abdominal Pain↗

PubMed2002

Related umbilical cord blood transplantation in patients with thalassemia and sickle cell disease.

Allogeneic bone marrow transplantation (BMT) from HLA-identical siblings is an accepted treatment for both thalassemia and sickle cell disease (SCD). However, it is associated with decided risk of both transplant-related mortality (TRM) and chronic graft-versus-host disease (GVHD). We analyzed 44 patients (median age, 5 years; range, 1-20 years) given an allogeneic related cord blood transplant for either thalassemia (n = 33) or SCD (n = 11). Thirty children were given cyclosporin A (CsA) alone as GVHD prophylaxis, 10 received CsA and methotrexate (MTX), and 4 patients received other combinations of immunosuppressive drugs. The median number of nucleated cells infused was 4.0 x 10(7)/kg (range, 1.2-10 x 10(7)/kg). No patient died and 36 of 44 children remain free of disease, with a median follow-up of 24 months (range, 4-76 months). Only one patient with SCD did not have sustained donor engraftment as compared with 7 of the 33 patients with thalassemia. Three of these 8 patients had sustained donor engraftment after BMT from the same donor. Four patients experienced grade 2 acute GVHD; only 2 of the 36 patients at risk developed limited chronic GVHD. The 2-year probability of event-free survival is 79% and 90% for patients with thalassemia and SCD, respectively. Use of MTX for GVHD prophylaxis was associated with a greater risk of treatment failure. Related CBT for hemoglobinopathies offers a good probability of success and is associated with a low risk of GVHD. Optimization of transplantation strategies could further improve these results.

Acute Disease↗

PubMed2002

Diminished antibody response to hepatitis B immunization in children with sickle cell disease.

Hepatitis B surface antibody titers were routinely measured in 150 children with sickle cell disease (SCD) after immunization, and the seroconversion rate was found to be lower than that in the general population (89% vs. 97%, P = 0.002). Most of the children who did not seroconvert after the series of 3 immunizations responded to booster injections (93%). Therefore, we recommend the measurement of hepatitis B surface antibody titers after immunization in those children with SCD at greatest risk for hepatitis B infection. An additional dose of hepatitis B vaccine should be administered to those without evidence of seroconversion.

Anemia, Sickle Cell↗

PubMedDate not supplied

Childhood non-Hodgkin lymphoma presenting as hemophagocytic syndrome.

An 8-year-old girl with a progressive systemic hemopagocytic syndrome was found to have non-Hodgkin lymphoma (NHL) after multiple nondiagnostic biopsies. Routine histochemistry and flow cytometry demonstrated this to be a peripheral T-cell process and cytogenetics identified a t(2;5)(p23;q35). An extensive evaluation for an infectious agent failed to identify a pathogen. Treatment according to a standard lymphoma protocol produced a rapid response and the girl remains in remission without evidence of hemophagocytic syndrome 18 months from diagnosis. In children with systenic hemophagocytosis, a diagnosis of NHL should be aggressively pursued.

Antineoplastic Combined Chemotherapy Protocols↗

PubMedDate not supplied

The autopsy in pediatrics and pediatric oncology: a single-institution experience.

Autopsy rates and clinicopathologic correlations for pediatric autopsies and the subgroup of pediatric oncology autopsies in a large teaching hospital were studied to evaluate the utility of autopsy in these populations. Autopsy records of the University of North Carolina hospitals from 1982 to 2001 were reviewed for all patients less than 18 years of age. Autopsies performed during 1982 to 1991 (decade 1) were compared to those from 1992 to 2001 (decade 2) with respect to absolute numbers of autopsy and rates of unexpected postmortem diagnoses. Postmortem diagnostic discrepancies were subclassified into major and minor categories. The mean number of autopsies per year for decade 1 was 110+/-24.5, compared with 77.5+/-40.9 for decade 2 (P<0.001), a change largely due to a decline in fetal/perinatal (patients<7 days of age) autopsies. Of 533 pediatric autopsies, 43 were in patients with a primary diagnosis of a neoplasia. At least one antemortem misdiagnosis and/or clinically occult process was identified in 20.5% of all pediatric autopsy cases, and in 25.6% of pediatric oncology cases. These rates did not change significantly over time. In 10 of 43 pediatric oncology cases (23.3%), autopsies provided diagnostic information that was previously unknown to the clinicians. Three of these patients died shortly after presentation. Although autopsy rates for fetal/perinatal cases have declined, those for non-perinatal and pediatric oncology patients remain stable. Autopsy continues to provide diagnostic information that is unknown during the antemortem period, and remains a valuable tool for pediatricians and pediatric oncologists.

Adolescent↗