Brief unconscious sedation for painful pediatric oncology procedures. Intravenous methohexital with appropriate monitoring is safe and effective.

PURPOSE: We report here our experience in using intravenous methohexital (MHX), an ultrashort-acting barbiturate, for brief unconscious sedation of pediatric oncology outpatients undergoing painful, invasive procedures. METHODS: Following published monitoring guidelines for deep pediatric sedation, 1.0 mg/kg MHX was administered immediately before the procedure, 1% xylocaine was given locally, and MHX was additionally titrated to maintain minimal response to pain during the procedure. Clinical data reported here were gathered retrospectively from permanent medical records. RESULTS: Data reported here represent 132 evaluable consecutive procedures in 33 patients ranging in age from 1.6 to 20.5 years. Patients underwent an average of 4 +/- 3 procedures and received a mean total MHX dose per procedure of 5.8 +/- 2.1 mg/kg. The mean length of time from start of sedation to full arousability was 30 +/- 12 min. Twenty-three (17.4%) procedures were associated with clinically insignificant decreases in diastolic blood pressure or heart rate below resting normal ranges for age. Eight (6.1%) procedures in six patients were associated with minor complications requiring no intervention, such as transient behavioral changes, transient myoclonus, and minimal stridor. Five procedures (3.8%) in five patients required simple suctioning to manage secretions. Only two procedures (1.5%) in two patients required brief bag-mask ventilation plus suctioning for suspected laryngospasm. None required intubation. No differences in clinical features or MHX doses were noted for patients with, as compared to those without, complications. All procedures were completed with a satisfactory level of sedation. CONCLUSIONS: Our experience indicates that MHX, with appropriate monitoring as described here, is a safe and effective agent for use in pediatric oncology outpatient sedation programs.

Extrarenal Wilms' tumor. Unusual presentation in the lumbosacral region.

True extrarenal Wilms' tumor is a rare malignant neoplasm most frequently presenting in the retroperitoneal or inguinal regions. We report an unusual subcutaneous lumbosacral (LS) region extrarenal Wilms' tumor without associated teratomatous tumor elements or associated neural tube defect in a 2 1/2-year-old girl. Pathologic review revealed features of true extrarenal Wilms' tumor, and the patient remains in complete remission following surgery and combination chemotherapy. This report illustrates the importance of early surgical intervention and pathologic examination of similar soft tissue masses in children.

Structural and functional basis of the developmental regulation of human coagulation factor IX gene: factor IX Leyden.

Hemophilia B Leyden is characterized by unusual developmental regulation of factor IX synthesis in affected individuals. One family affected with the hemophilia B Leyden phenotype was found to have a specific single-base mutation (G----A) at nucleotide -6 of the factor IX gene. The mutation site was found in a small region of the 5'-untranslated sequence designated the Leyden-specific region (LS region). This region, approximately 40 base pairs in length, contains the unique mutation sites of all the known factor IX Leyden genes (five families) analyzed to date. This fact strongly suggests that the LS region is directly or indirectly involved in the developmental regulation of factor IX biosynthesis. Base changes at nucleotide -20 as well as at nucleotide -6 and deletions of the 3' half of the LS region reduced expression activity of the factor IX gene to approximately 15-31% that of the normal control, as assessed in a cultured cell (HepG2) expression system. The LS region binds at least two proteins. Androgen significantly increased the transcriptional activities of both mutant and normal factor IX genes in a concentration-dependent manner.