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Biomedical subjects

Susan R Rheingold

Publications and source records attributed to Susan R Rheingold.

8 recordsLinked to original sources
PubMed2007

Acute myeloid leukemia in a child with hereditary thrombocytopenia.

A child with a known diagnosis of an autosomal dominant macrothrombocytopenia, Fechtner Syndrome, developed acute myeloid leukemia (AML). Recently the disease gene for the inherited macrothrombocytopenias has been identified as MYH9, encoding for non-muscle myosin heavy chain-A. MYH9 has never been associated with the development of acute leukemia, but MYH11 is disrupted in the M4 eosinophilia sub-type of AML (inv16). The patients leukemic blasts did carry the common t(8;21) which yields an AML1-ETO fusion protein that inhibits AML-1. Despite his thrombocytopenia, the patient successfully completed intensive bone marrow cytoreduction without significant bleeding complications and is now in remission for over 3 years.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2006

A child with anterior mediastinal mass supported with veno-arterial extracorporeal membrane oxygenation.

OBJECTIVE: To demonstrate the utility of rescue with veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and to demonstrate the feasibility of administration of chemotherapy during VA-ECMO in a child with an anterior mediastinal mass. DESIGN: Case report. SETTING: Large, tertiary care, pediatric intensive care unit. PATIENT: One patient with cardiopulmonary collapse in the setting of a new diagnosis of an anterior mediastinal mass. INTERVENTIONS: Cardiopulmonary support with VA-ECMO; administration of chemotherapy during VA-ECMO. MEASUREMENTS AND MAIN RESULTS: Successful rescue with VA-ECMO and successful chemotherapy while on VA-ECMO. CONCLUSIONS: VA-ECMO can be successfully used to support patients with cardiopulmonary failure during initial diagnosis of an anterior mediastinal mass. In addition, we have demonstrated that successful chemotherapy can be administered while the patient is supported on VA-ECMO.

Antineoplastic Agents↗
PubMed2005

Antioxidant status decreases in children with acute lymphoblastic leukemia during the first six months of chemotherapy treatment.

BACKGROUND: Children undergoing treatment for acute lymphoblastic leukemia (ALL) receive combination chemotherapy and many of the components are associated with free radical production. PROCEDURE: Among 103 children newly diagnosed with ALL, plasma concentrations of antioxidants, total antioxidant capacity (ORAC), and DNA oxidized base 8-oxodeoxyguanosine (8-oxo-dG) were analyzed at baseline and 3 and 6 months after diagnosis. RESULTS: Plasma vitamin A, antioxidants, 8-oxo-dG, and ORAC changed from diagnosis through the first 6 months of ALL therapy. In patients with higher plasma concentrations of vitamin A, E, total carotenoids, ORAC, and 8-oxo-dG there was a beneficial association with fewer dose reductions, fewer infections, improved quality of life, less delay in chemotherapy treatment schedule, reduced toxicity, and fewer days spent in the hospital. There were also adverse relationships demonstrated. CONCLUSIONS: Among children with ALL, antioxidant levels and oxidative stress appear to be associated with duration and complications of treatment.

8-Hydroxy-2'-Deoxyguanosine↗
PubMed2004

Low antioxidant vitamin intakes are associated with increases in adverse effects of chemotherapy in children with acute lymphoblastic leukemia.

BACKGROUND: Chemotherapy leads to an increase in reactive oxygen species, which stresses the antioxidant defense system. Children with acute lymphoblastic leukemia rarely are overtly malnourished, which makes this population ideal for an investigation of the relations between dietary antioxidant consumption, plasma antioxidant concentrations, and chemotherapy-induced toxicity. OBJECTIVE: This study was conducted to investigate the effect of therapy on antioxidant intakes in children with acute lymphoblastic leukemia, the relation between dietary antioxidant intakes and plasma antioxidant concentrations, and the relation between the incidence of side effects due to treatment and antioxidant intake. DESIGN: We conducted a 6-mo observational study of 103 children with acute lymphoblastic leukemia. Plasma micronutrient concentrations, dietary intakes, and incidence of side effects of chemotherapy were ascertained at diagnosis and after 3 and 6 mo of therapy. RESULTS: Throughout the 6-mo study period, subjects ingested vitamin E, total carotenoid, beta-carotene, and vitamin A in amounts that were 66%, 30%, 59%, and 29%, respectively, of the US recommended dietary allowance or of the amounts specified in the third National Health and Nutrition Examination Survey. Greater vitamin C intakes at 6 mo were associated with fewer therapy delays, less toxicity, and fewer days spent in the hospital. Greater vitamin E intakes at 3 mo were associated with a lower incidence of infection. Greater beta-carotene intakes at 6 mo were associated with a decreased risk of toxicity. CONCLUSION: A large percentage of children undergoing treatment for acute lymphoblastic leukemia have inadequate intakes of antioxidants and vitamin A. Lower intakes of antioxidants are associated with increases in the adverse side effects of chemotherapy.

Adolescent↗
PubMed2004

HIV infection presenting as severe autoimmune hemolytic anemia with disseminated intravascular coagulation in an infant.

Autoimmune hemolytic anemia is rare in children. It is generally diagnosed in relation to a viral or bacterial infection but has also been described in association with drugs, autoimmune disease, malignancy, and immunodeficiency. The authors describe a 5-month-old infant who presented with severe autoimmune hemolytic anemia and disseminated intravascular coagulation as his initial manifestation of HIV infection.

Anemia, Hemolytic, Autoimmune↗
PubMed2003

Role of the BCR complex in B cell development, activation, and leukemic transformation.

A primary focus of signal transduction in B cells, from the pre-B cell to the mature B cell, is the B cell receptor complex. Here we describe work demonstrating the importance of signaling via the pre-B cell receptor complex (pre-BCR) to the pre-B cell transition, the central checkpoint in B-cell development. We have shown tht pre-BCR complex components Igalpha and Igbeta are critical to allowing the pre-B cell to move through this transition, but may not be required for allelic exclusion. Pre-BCR expression also directly affects the response of leukemic cells to steroid treatment, suggesting that signals initiated by the pre-BCR complex may present therapeutic targets in acute leukemia. Additionally, interleukin-7 may also modulate the response of leukemic cells arising from early B-cell stages to treatment. This observation has lead directly to proposals to test drugs which may antagonize early B-cell growth signals, such as rapamycin, in acute lymphoid leukemia.

Animals↗
PubMedDate not supplied

A childhood cavernous conundrum.

A 9-year-old boy presented with a left-sided "tooth ache." Subsequently, he developed new onset vertical diplopia. He was found to have a left cranial nerve three palsy. Cranial magnetic resonance imaging showed bilateral cavernous sinus lesions that were initially interpreted as possible "cavernous sinus thrombosis." Subsequent blood smear and bone marrow biopsy disclosed Burkitt lymphoma. He was treated with chemotherapy and made a dramatic recovery. Burkitt lymphoma rarely presents with a third nerve palsy but should be considered in the differential diagnosis of childhood cavernous sinus syndrome.

Antineoplastic Combined Chemotherapy Protocols↗