Sue McIntosh

Chronic neurologic disturbance in childhood leukemia.

Twenty‐three leukemic children were studied prospectively to detect chronic effects of therapy. All patients received CNS prophylaxis, including 2400 R cranial irradiation, and intermittent maintenance therapy with intravenous methotrexate, cyclophosphamide and cytosine arabinoside. Neurologic symptoms were observed in 12 patients, all of whom had intermittent limping and mild incoordination, between the 10th and 18th month of maintenance therapy. Five of the 12 sustained seizures and four of these had subsequent abnormalities in motor, perceptual, behavioral or language development. Three school‐aged children have learning disability and perceptual‐motor defects. Studies of CSF folate and MTX content are presented but are not helpful in delineating the etiology of these neurologic symptoms.

Intracranial calcifications in childhood leukemia: An association with systemic chemotherapy*

Children with acute lymphocytic leukemia were examined for evidence of intracranial calcifications withroentgenograms of the skull and computerized tomography. Of 39 children in their initial complete remission, ten were found to have subcortical cerebral calcifications. Significant associations were found between the presence of cerebral calcifications and systemic treatment with large cumulative doses of methotrexate.

Ovarian germ cell malignancies: The Yale University experience

Abstract Eighty-one patients with ovarian germ cell malignancies (immature teratoma 29, dysgerminoma 26, endodermal sinus tumors 15, mixed germ cell tumor 8, other 3) seen or consulted on at Yale University over a 15 year period are presented. Initial therapy was successful in 70 of 81 (86.4%) patients and 75 (92.6%) are currently alive and disease free. Early stage dysgerminomas may be safely treated with surgery whereas advanced disease is exquisitely sensitive to vincristine, actinomycin D, and cyclophosphamide (VAC) therapy. Early stage immature teratoma is uniformly successfully treated with short-term VAC whereas advanced disease requires longer treatment. Early stage endodermal sinus tumor (EST) and mixed germ cell tumors may be effectively treated with VAC or platinum-based therapy but advanced disease should be treated with paltinum-based regimens. Serial alpha fetoprotein assays should determine duration of therapy in tumors containing EST elements. Conservative surgery to preserve reproductive function is appropriate for all patients with early stage ovarian germ cell malignancies and selected patients with advanced disease.

Prospective study of sickle cell anemia in infancy.

Twelve infants with sickle cell anemia identified in the course of a cord blood screening program have been followed prospectively for up to three years of age. The development of hemolytic anemia paralleled the postnatal decline in fetal hemoglobin and was evident in all infants by 12 weeks of age. Vasoocclusive episodes occurred in more than half the infants and seven aplastic crises were documented in four patients. Febrile illnesses were common and one of the twelve infants developed pneumococcal sepsis. This study also demonstrated that functional asplenia is an acquired defect in sickle cell disease. The onset of functional asplenia was documented with splenic scans in six of the nine infants followed for more than one year after birth. There have been no deaths in this series.

Screening for thalassemia trait by electronic measurement of mean corpuscular volume.

THALASSEMIAS are a group of hereditary blood conditions with clinical and genetic implications that occur frequently in Mediterranean ethnic groups. There have been few attempts to screen high-prev...

Fever in young children with sickle cell disease.

Bacterial septicemia and meningitis are major causes of death in young children with sickle cell anemia. We report a prospective study of 182 episodes of fever among 22 children with sickle cell diseases identified at birth. The majority of patients with fever exceeding 39.5 degrees C were treated with antibiotics intravenously until results of blood cultures were known. Infectious diseases were documented in 38% of all febrile events. Six episodes of bacterial septicemia, two associated with meningitis, were successfully treated with no mortality or residual morbidity. These data form the basis of suggestions for management of the young child with sickle cell disease and fever.

Adjuvant chemotherapy for medulloblastoma

The use of adjuvant chemotherapy for cerebellar medulloblastoma is controversial. Twenty‐one children and adolescents were treated with adjuvant low‐dose cyclophosphamide and vincristine following surgery and radiotherapy. With a mean observation period of 6 years, the disease‐free survival is 81%.

Methotrexate hepatotoxicity in children with leukemia.

Percutaneous liver biopsy was performed on seven children with acute lymphocytic leukemia who had received maintanance chemotherapy with high-dose intravenous methotrexate for 1 1/2 to 2 1/2 years. Portal fibrosis was found in four of the seven children and in an additional patient treated with oral methotrexate. Serial tests of liver function and 99mtechnetium-sulfur-colloid scans did not accurately identify children with hepatic fibrosis. Clinical evidence of liver disease was not seen.

Neonatal isoimmune purpura: Response to platelet infusions

Neonatal isoimmune thrombocytopenia is a self-limited disease of antigenic incompatibility and platelet destruction caused by transplacentally acquired maternal antibody. Because of a mortality rate of 10 to 15 per cent due to hemorrhage initiated by the birth process, immediate therapy is advocated. Infusion of compatible platelets obtained from the mother is a safe therapeutic procedure which also helps to confirm the diagnosis. When compatible platelets cannot be obtained, exchange transfusion is the recommended treatment. Corticosteroids also appear to be beneficial, but few controlled studies are available. In milder forms of thrombocytopenia, isoimmune disease can usually be differentiated from other causes of neonatal purpura by comparing survival of maternal platelets to those obtained from the father or an incompatible random donor.

Cytophilic Antibodies in Man

Abstract Antibodies cytophilic for macrophages, mediating attachment of immunologically naive macrophages to leukemic cells, were found in the serum of 25 patients with acute myelocytic or lymphocy...