Peter Waldron

Successful treatment of a patient with stage IV rhabdoid tumor of the kidney: case report and review.

The clinical course of a 31-month-old patient with advanced (stage IV) rhabdoid tumor of the kidney (RTK) and an analysis of treatment variables that may impact survival are presented. Treatment included complete resection of abdominal disease, radiation therapy to the abdomen and chest, and chemotherapy on a schedule of dose intensification by reduction of the interval between cycles. Inclusion of doxorubicin in treatment was associated with survival among patients in published series (P = 0.002). The patient was in continuous complete remission 60 months from diagnosis. Stage IV rhabdoid tumor of the kidney can be effectively treated with intensive multimodal therapy. Doxorubicin may be an important component of a successful therapeutic regimen.

Tonsillectomy, Adenoidectomy, and Myringotomy in Sickle Cell Disease: Perioperative Morbidity

PURPOSE To compare the rates of perioperative morbidity of patients with sickle cell anemia who were randomly assigned to 2 preoperative transfusion regimens and to identify predisposing factors for perioperative complications. PATIENTS AND METHODS Investigators at 36 centers enrolled 118 patients who were scheduled to have elective surgery and agreed to randomization between 2 preoperative transfusion regimens. Forty-seven subjects were enrolled but not randomized, including 20 who were not transfused before surgery. Perioperative management was based on a prescribed care plan. RESULTS Tonsillectomy and/or adenoidectomy (TA) were performed on 136 persons, and 29 had myringotomy as their primary procedure. There were no differences in the frequency of complications between the randomized groups. The serious, non-transfusion complication rates for randomized patients were 32% (34 of 107) for TA and 36% (4 of 11) for myringotomy. A history of pulmonary disease was a predictor of postoperative sickle cell-related events for patients undergoing TA surgery. CONCLUSIONS The more intensive transfusion regimen did not result in fewer perioperative complications. The high frequency of complications emphasizes the need for anticipatory management of persons undergoing TA. A history of pulmonary disease identifies patients at increased risk for sickle cell-related events after TA surgery. Patients undergoing myringotomy have a low frequency of sickle cell-related events but a significant frequency of other serious perioperative complications.

Chlamydia pneumoniae and acute chest syndrome in patients with sickle cell disease

Purpose Few studies address the association of Chlamydia pneumoniae infection with pulmonary disease and outcome in patients with underlying pathology such as sickle cell disease (SCD). SCD patients are susceptible to the pulmonary disorder known as acute chest syndrome (ACS), where the etiology remains ill defined. The purpose of this study was to analyze the clinical course and outcome of C. pneumoniae-associated ACS among SCD patients as part of the National Acute Chest Syndrome Study. Patients and Methods This was a longitudinal study of SCD patients presenting with ACS to multiple U.S. medical centers. Two hundred ninety-six SCD patients who developed ACS were tested by PCR for C. pneumoniae and by standard techniques for other respiratory pathogens. These infections were evaluated for association with ACS, clinical course, and complications. Results Forty-one (14%) patients with first episodes of ACS were PCR positive for C. pneumoniae. Compared with other infections, C. pneumoniae-infected patients were older, were more likely to present with chest pain, and had higher hemoglobin levels at diagnosis. Both groups had similar rates of respiratory failure and prolonged hospitalization. Of the 89 patients with single-pathogen infections, 27 (30%) were due to C. pneumoniae, 21% to Mycoplasma pneumoniae, 10% to RSV, 4% to Staphylococcus aureus, and 3% to Streptococcus pneumoniae. Conclusions C. pneumoniae was the most prevalent pathogen in this study of ACS and was responsible for significant morbidity. Additional research is required to develop effective treatment guidelines for ACS.

Successful Management of Stage 4s Neuroblastoma and Severe Hepatomegaly Using Absorbable Mesh in an Infant

This report describes the use of an absorbable mesh in an infant with stage 4S neuroblastoma who required decompressive laparotomy. At the time of laparotomy, a SILASTIC silo was placed. After 12 days, the liver had not reduced in size despite chemotherapy and radiation therapy. Because of concern for infection, the silo was removed, and an absorbable polygalactin (Vicryl) mesh was placed. Wet-to-dry dressings were used to manage the mesh. A granulation base developed that provided a physiological closure of the abdominal cavity. Forty-two days after placement of the absorbable mesh, the liver had reduced to a size that permitted mobilization of skin flaps for a surgical abdominal closure. The liver continued to reduce in size, allowing the fascial edges to draw together. The patient is now 2 years old with no signs of residual tumor or ventral hernia.

Interferon treatment of chronic active hepatitis C during therapy of acute lymphoblastic leukemia

Due to concerns that antineoplastic therapy produces prolonged decrease in immune function, interferon treatment of chronic active hepatitis C (CAHC) has been used only at one year or longer after the end of cancer therapy. We report the experience of an 11‐year‐old who developed symptomatic CAHC at the start of maintenance therapy for testicular relapse of acute lymphoblastic leukemia (ALL). Significant dose reduction of maintenance therapy did not improve the tolerance of antileukemic treatment. In an effort to improve his liver disease and to deliver effective antileukemic therapy, interferon alpha and an alternative maintenance therapy regimen for ALL were initiated. The patient tolerated the combined therapy well. Interferon therapy was continued for 27 months, which was three months from the end of antineoplastic therapy. At that time serum transaminase values were normal, and no HCV viral genome was detectable. Viral genome was detected 10 months later. The combined effects of interferon and antineoplastic therapy resulted in myelosuppression requiring dose reduction of both treatments. The patient remains asymptomatic and with no evidence of recurrent leukemia more than six years from diagnosis of relapse. The effect on the status of this patients CAHC was similar to that reported among leukemic patients who underwent an interferon course more than one year from the end of antineoplastic therapy. Interferon treatment of CAHC can be given concomitantly with antineoplastic therapy. Am. J. Hematol. 61:130–134, 1999.

The Use of Activated Factor VII for Ventricular Septal Defect Closure in a Pediatric Patient With Hemophilia A and a High Titer of Inhibitor

A C EMOPHILIA A IS A bleeding diathesis caused by factor VIII (FVIII) deficiency. It may be complicated by inhibtors which are auto antibodies to FVIII.1 Recombinant actiated factor VII (rFVIIa) is a potent hemostatic agent approved y the Food and Drug Administration for use in hemophiliacs ith inhibitors. A case in which rFVIIa was used for the uccessful closure of a ventricular septal defect in a patient with emophilia A and a high titer inhibitor is presented.