Faculty Bibliography

Prevalence of posttraumatic stress disorder (PTSD) in 24 mothers of pediatric cancer survivors was compared with its prevalence among 23 mothers of healthy children. Significantly more mothers of pediatric cancer survivors were diagnosed with lifetime PTSD. Significant differences were also found in lifetime arousal, as well as current and lifetime reexperience and avoidance symptom clusters. Significant difference existed in the distribution of the number of prediagnosis high-magnitude events experienced by the mothers diagnosed with current PTSD as compared with the prediagnosis experience of the mothers who were not diagnosed with current PTSD. Illness severity, level of perceived family and extrafamilial social support, and Symptom Checklist-90-Revised global severity index scores did not significantly differ in the PTSD-positive and PTSD-negative groups

In an effort to simplify chemotherapy dose calculations and reduce the incidence of medication errors, a commercially available, general database management program was used to aid in the management of an active pediatric hematology/oncology service. The software was customized to include all medications used by the specialty practice, as well as some used in a general pediatric setting. After entering a patient's height and weight, the database rapidly computes and immediately displays the patient's surface area, appropriate intravenous fluid rates, and doses of antibiotic, chemotherapeutic, and other drugs. No prior computer expertise is required to run the program, which can be used easily by physicians and nurses. A database program similar to this can be implemented and customized easily for any hematology/oncology practice, and can facilitate patient care by reducing both the time and effort needed to order medications as well as the number of medication errors

A cytogenetically normal infant with Kostmann syndrome (severe congenital granulocytopenia) was treated with granulocyte colony-stimulating factor, which resulted in a rapid improvement in his neutrophil count and a resolution of recurrent infections. After 11 months of therapy, splenomegaly developed, with thrombocytopenia, anemia, circulating nucleated erythrocytes, and acquired monosomy 7, which evolved during a period of 7 months into acute nonlymphoblastic leukemia. The use of granulocyte colony-stimulating factor in patients with congenital marrow failure disorders may induce or hasten the onset of a malignant transformation

A fetus with absent radii in both forearms was discovered on routine ultrasound examination performed at 18 weeks of pregnancy. No other significant abnormalities were found, and no signs of haemorrhage were detected. Serial ultrasound examinations revealed no evidence of fetal internal bleeding. At 37 weeks of pregnancy, a CBC obtained by cordocentesis under ultrasound guidance confirmed the diagnosis of thrombocytopenia absent radii (TAR) syndrome. Apheresis platelets were transfused into the umbilical vein to correct the thrombocytopenia and was followed by an uncomplicated delivery. No bleeding was encountered during the remainder of the baby's neonatal course. We conclude that TAR syndrome can be readily identified prenatally on sonogram, and if severe thrombocytopenia is confirmed by cordocentesis, platelets should be transfused to diminish the risk of serious internal bleeding during and immediately after delivery

A severely anemic 3-year-old girl with refractory sideroblastic anemia and fulminant, fatal hemochromatosis is described. The patient had transfusion-dependent anemia with clinical cardiac, liver, and endocrine dysfunction that resulted from iron loading. The patient was minimally transfused, and deferoxamine chelation was started at age 34 months. Despite treatment, the patient died at age 46 months as a result of severe iron overload. Sideroblastic anemia and iron overload in childhood are reviewed, and a pathophysiologic mechanism for the patient's clinical course is postulated

A patient with acute lymphoblastic leukemia (ALL) who had undergone an allogeneic bone marrow transplant that included high-dose chemotherapy and total body radiation without eye shielding, sustained an isolated relapse of her disease in the iris. A review of ocular leukemic disease is discussed

An unusual case of Diamond-Blackfan syndrome whose initial presentation was hydrops fetalis is presented. Diamond-Blackfan syndrome and the pathophysiology of hydrops fetalis in severely anemic infants are briefly reviewed

An acutely ill 4-year-old girl with Rocky Mountain spotted fever (RMSF) was found to have a coagulation inhibitor. This child had no serious bleeding manifestations and minimal hemorrhagic skin manifestations despite severe RMSF, concurrent thrombocytopenia, as well as the coagulation inhibitor. Hemostatic abnormalities that occur with RMSF as well as other infectious illnesses associated with coagulation inhibitors are reviewed