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Biomedical subjects

Deborah F Billmire

Publications and source records attributed to Deborah F Billmire.

12 recordsLinked to original sources

Incidence of persistent left superior vena cava in esophageal atresia.

PURPOSE: Esophageal atresia is known to be associated with a variety of additional congenital anomalies in multiple organ systems. Emphasis on cardiovascular anomalies has been focused on aortic arch and intrinsic cardiac malformations. Persistent left superior vena cava (PLSVC) is the most common venous thoracic anomaly in the general population and creates a problem when central venous access is required. This review was undertaken to define the incidence of PLSVC in infants with esophageal atresia and to determine if any subgroup of associated anomalies poses additional risk. METHODS: A retrospective, institutional review board-approved review of all children treated for esophageal atresia from 1993 to 2002 at Riley Hospital for Children was undertaken. Of 118 children, 89 had sufficient data for inclusion. Charts were reviewed for gestational age, weight, type of atresia, echocardiogram, and associated anomalies. Statistical analysis was performed using the Fisher's Exact test. RESULTS: Of 89 children, 8 (9.9%; confidence interval, 4%-17%) had PLSVC compared with the reported incidence of 0.3% in the general population. Presence of additional organ system anomalies did not significantly increase relative risk for PLSVC. CONCLUSION: The incidence of PLSVC is significantly increased in children with esophageal atresia when compared with the general population. This increased incidence of PLSVC is not influenced by the presence of cardiac or other associated anomalies. This finding should be kept in mind when central venous access is required in this patient population.

Abnormalities, Multiple↗

Malignant germ cell tumors in childhood.

Malignant germ cell tumors are a very heterogeneous group of neoplasms that have historically carried a poor prognosis. A review of the cooperative studies on malignant extracranial germ cell tumors in children was performed to update the progress in treatment and outcome for this rare group of tumors. The advent of platinum-based chemotherapy in the 1980s dramatically improved the prognosis for these tumors and allowed redefinition of risk groups. Chemotherapy regimens have been sequentially modified to reduce toxicity while maintaining survival. Surgical management has evolved from an aggressive en bloc resection at diagnosis to a more tailored approach, with resection and meticulous staging for low stage tumors at diagnosis and biopsy with neoadjuvant chemotherapy for advanced stage tumors. Improved surgical margins and prognosis are seen in post chemotherapy resections for high stage tumors. Large persisting masses after chemotherapy are often due to coexisting elements of benign teratoma, and aggressive attempts at resection are warranted in these patients with expectation of successful outcome.

Child↗

Amifostine does not protect against the ototoxicity of high-dose cisplatin combined with etoposide and bleomycin in pediatric germ-cell tumors: a Children's Oncology Group study.

BACKGROUND: High-dose cisplatin combined with etoposide and bleomycin (HDPEB) improves event-free survival (EFS) in advanced pediatric germ-cell tumors (PGCT), but has significant ototoxicity. Amifostine appears to protect against toxicity. The authors combined amifostine with HDPEB and evaluated the efficacy and toxicity, specifically whether ototoxicity decreased. METHODS: Eligibility criteria included age < 15 years and unresectable Stage III/IV extracranial, extragonadal PGCT. Patients received bleomycin 15 IU/m(2) on Day 1, then etoposide 100 mg/m(2) per day, amifostine 825 mg/m(2) per day, and cisplatin 40 mg/m(2)per day on Days 1-5, intravenously. The cycles were repeated every 3-4 weeks with imaging evaluation after 4 cycles. Patients with residual radiographic abnormalities underwent resection. Patients with residual tumor received two additional HDPEB cycles. Hearing evaluations were required at diagnosis and after two and four cycles. Audiologic results were reviewed and compared with historical controls treated with HDPEB. RESULTS: Twenty-five eligible patients were enrolled between April 2000 and April 2002. Their median age was 1.6 years (range, 0.64-13.9 years), 17 patients were female, 11 had metastases, and 24 had a yolk sac carcinoma component histologically. Primary sites included sacrococcygeal area/pelvis (n = 15), vagina (n = 5), and other (n = 5). Two-year EFS and overall survival were 83.5% +/- 12.8% and 85.6% +/- 12.3%, respectively. Eight patients were removed from the study (four had progressive disease/disease recurrence and four had ototoxicity). Grade 3/4 toxicities included neutropenia (n = 20), thrombocytopenia (n = 14), electrolyte imbalances (n = 14), and gastrointestinal toxicity (n = 12). Twenty-four of 25 patients received hearing evaluations, and 75% had significant hearing loss. CONCLUSIONS: Amifostine did not protect against HDPEB-associated ototoxicity.

Adolescent↗

Appendicitis in childhood hematologic malignancies: analysis and comparison with typhilitis.

BACKGROUND/PURPOSE: Recognition of appendicitis in the child with hematologic malignancy may be difficult particularly in the setting of neutropenia and multiple medications causing an altered inflammatory response. Typhilitis may produce a similar constellation of clinical findings causing further diagnostic confusion. This review compares the relative frequency of these two conditions in children with hematologic malignancy with a focus on the clinical presentation, distinguishing features, surgical management, and outcome for patients with appendicitis. METHODS: This institutional review board-approved retrospective study evaluated 464 pediatric patients treated for hematologic malignancy at our institution from 1997 to 2003. From this cohort, we identified all children with a diagnosis of appendicitis or typhilitis. Data include demographics, clinical presentation, laboratory studies, and computed tomography (CT) scan findings. Groups were compared using the Fisher exact test. Significance was defined as P < .05. RESULTS: Eight (1.7%) of 464 children were diagnosed with typhilitis and 7 (1.5%) with appendicitis. There were no demographic differences between patients with appendicitis and typhilitis. Distinguishing clinical features in children with typhilitis included presence of fever and diarrhea. Clinical presentation in children with appendicitis was atypical in 5 of 7 cases yielding an incorrect preoperative diagnosis in all 5. Radiographic evaluation by CT scan accurately defined typhilitis, but not appendicitis. An operation was performed on all 7 children with appendicitis with no operative morbidity or mortality. CONCLUSIONS: Appendicitis and typhilitis occur with similar frequency in children with leukemia and lymphoma. Typhilitis is accurately diagnosed with clinical findings of fever, diarrhea, abdominal pain, and typical CT scan findings. Appendicitis tends to present with atypical findings, but can be successfully managed with standard surgical care.

Adolescent↗

Supraumbilical pyloromyotomy: a unique indication for antimicrobial prophylaxis.

BACKGROUND: The umbilical fold incision for infantile hypertrophic pyloric stenosis provides a convenient exposure and cosmetically appealing scar. This study investigates the possible difference in infection rates between traditional and supraumbilical approaches for pyloromyotomy. METHODS: All patients who underwent pyloromyotomy for infantile hypertrophic pyloric stenosis at a tertiary pediatric hospital were reviewed. Baseline wound infection rate was determined through review of patients with right upper quadrant incisions (group 1). A nonrandomized comparison was performed between patients with a supraumbilical approach (group 2) and those undergoing supraumbilical incisions after prophylactic antibiotic administration (group 3). RESULTS: Complete records were reviewed on 384 patients over a 6-year period. Demographics and preoperative factors were similar among groups. The rate of infection in group 1 (n = 258) was 2.3%. With introduction of the supraumbilical approach, there was a statistically significant increase in wound infection rate to 7.0% (chi 2 ; group 1 vs group 2, P < .05). The use of prophylactic antibiotics with a supraumbilical approach reduced this rate of infection back to 2.3% (chi 2 ; group 1 vs group 3, P < 1.0 and group 2 [n = 85] vs group 3 [n = 42], P < .3). CONCLUSIONS: The risk of wound infection by classic pyloromyotomy of 2.3% is significantly increased with an open supraumbilical approach. The use of prophylactic antibiotics reduces this risk of wound infection.

Anti-Infective Agents↗

Surgical considerations in cystic fibrosis: a 32-year evaluation of outcomes.

BACKGROUND: Information concerning long-term operative outcomes in patients with cystic fibrosis (CF) is relatively sparse in the operative literature. METHODS: A retrospective review of CF patients with operative conditions was performed (1972-2004) at a tertiary children's hospital to analyze outcomes including long-term morbidity and survival. RESULTS: A total of 226 patients with CF presented with an operative diagnosis (113 men, 113 women). A total of 422 operations were performed in 213 patients (94%). The mean age at operation was 4.1 +/- 6.2 years (range, 1 d to 26 y) and 109 were neonates. Fifteen of 42 (36%) babies with simple meconium ileus (MI) were treated nonoperatively with hypertonic enemas, 27 of 42 and all 45 patients with complicated MI required operation, including 15 with jejunoileal atresia (17%). Seventeen of 27 (63%) patients with meconium ileus equivalent had MI as neonates; 7 of 27 (26%) required operation. Eight of 9 (89%) with fibrosing colonopathy required operation. Organ transplantation was required in 21 patients. Follow-up evaluation was possible in 204 of 213 (96%) patients. The duration of follow-up evaluation was 14.9 +/- 8.5 years (range, 2 mo to 35 y). Operative morbidity was 11% at 1 year, 2% at 2 to 4 years, 1% at 5 to 10 years, and less than 1% at more than 10 years. There were 24 deaths (11%); 22 followed CF-related pulmonary complications and included 8 of 16 (50%) children with pneumothorax. CONCLUSIONS: Long-term survival in CF patients has improved significantly (89%), with many surviving into the fourth decade. MI may predispose to late complications including meconium ileus equivalent and fibrosing colonopathy. Pneumothorax in CF patients is an ominous predictor of mortality. Children with CF are living longer and are good candidates for operation, but require long-term follow-up evaluation because of ongoing exocrine dysfunction.

Abdomen↗

Treatment of children and adolescents with stage II testicular and stages I and II ovarian malignant germ cell tumors: A Pediatric Intergroup Study--Pediatric Oncology Group 9048 and Children's Cancer Group 8891.

PURPOSE: To determine whether children with localized gonadal malignant germ cell tumors (MGCT) stage II testicular and stages I and II ovarian treated with four cycles of standard-dose cisplatin combined with etoposide and low-dose bleomycin (PEB) have an event-free survival (EFS) of at least 85% without significant toxicity. PATIENTS AND METHODS: Between May 1990 and July 1995, eligible pediatric patients with stage II or recurrent from stage I (as a stage II) testicular MGCT and stages I and II ovarian MGCT were enrolled onto this Pediatric Oncology Group and Children's Cancer Group study. PEB chemotherapy consisted of bleomycin 15 U/m2 on day 1, cisplatin 20 mg/m2/d on days 1 to 5, and etoposide 100 mg/m2/d on days 1 to 5. Patients received four cycles of therapy at 21-day intervals. RESULTS: Seventy-four patients with a median age of 10.5 years (range, 8.7 months to 16.7 years) were enrolled. Primary sites included: stage II testicular (n = 17), stage I ovarian (n = 41), and stage II ovarian MGCT (n = 16). Treatment with standard PEB resulted in 6-year EFS of 95% and overall survival (OS) of 95.7%. EFS and OS by primary site were as follows: stage II testicular, 100% and 100%; stage I ovarian, 95.1% and 95.1%; and stage II ovarian, 87.5% and 93.8%, respectively. Two patients died from recurrent disease, and one patient died of secondary acute myelocytic leukemia. Infrequent grade 3 to 4 hematologic toxicity was reported. No grade 3 to 4 renal, pulmonary, or ototoxicity was observed. CONCLUSION: Combination chemotherapy with PEB results in excellent EFS and OS with minimal toxicity in children and adolescents with localized gonadal MGCT.

Adolescent↗

Randomized comparison of combination chemotherapy with etoposide, bleomycin, and either high-dose or standard-dose cisplatin in children and adolescents with high-risk malignant germ cell tumors: a pediatric intergroup study--Pediatric Oncology Group 9049 and Children's Cancer Group 8882.

PURPOSE: To determine in a randomized comparison whether combination chemotherapy with high-dose cisplatin (HDPEB) improves the event-free (EFS) and overall (OS) survival of children and adolescents with high-risk malignant germ cell tumors (MGCT) as compared with standard-dose cisplatin (PEB) and to compare the regimens' toxicity. PATIENTS AND METHODS: Between March 1990 and February 1996, 299 eligible patients with stage III and IV gonadal and extragonadal (all stages) MGCT were enrolled onto this Pediatric Oncology Group and Children's Cancer Group study. Chemotherapy included bleomycin 15 units/m(2) on day 1, etoposide 100 mg/m(2) on days 1 through 5, and either high-dose cisplatin 40 mg/m(2) on days 1 through 5 (HDPEB; n = 149) or standard-dose cisplatin 20 mg/m(2) on days 1 through 5 (PEB; n = 150). Patients were evaluated after four cycles of therapy, and those with residual disease underwent surgery. Those with malignant disease in resected specimen received two additional cycles of their assigned regimen. RESULTS: One hundred thirty-four eligible patients with advanced testicular (n = 60) or ovarian (n = 74) tumors and 165 with stage I to IV extragonadal tumors were enrolled. HDPEB treatment resulted in significantly improved 6-year EFS rate +/- SE (89.6% +/- 3.6% v 80.5% +/- 4.8% for PEB; P =.0284). There was no significant difference in OS (HDPEB 91.7% +/- 3.3% v PEB 86.0% +/- 4.1%). Tumor-related deaths were more common after PEB (14 deaths v two deaths). Toxic deaths were more common with HDPEB (six deaths v one death). Other treatment-related toxicities were more common with HDPEB. CONCLUSION: Combination chemotherapy with HDPEB significantly improves EFS for children with high-risk MGCT. The OS is similar in both regimens, and the significant toxicity associated with HDPEB limits its use.

Adolescent↗

Duodenal atresia and stenosis: long-term follow-up over 30 years.

BACKGROUND: Duodenal atresia and stenosis is a frequent cause of congenital, intestinal obstruction. Current operative techniques and contemporary neonatal critical care result in a 5% morbidity and mortality rate, with late complications not uncommon, but unknown to short-term follow-up. METHODS: A retrospective review of patients with duodenal anomalies was performed from 1972 to 2001 at a tertiary, children's hospital to identify late morbidity and mortality. RESULTS: Duodenal atresia or stenosis was identified in 169 patients. Twenty children required additional abdominal operations after their initial repair with average follow-up of 6 years (range, 1 month to 18 years) including fundoplication (13), operation for complicated peptic ulcer disease (4), and adhesiolysis (4). Sixteen children underwent revision of their initial repair: tapering duodenoplasty or duodenal plication (7), conversion of duodenojejunostomy to duodenoduodenostomy (3), redo duodenojejunostomy (3), redo duodenoduodenostomy (2), and conversion of gastrojejunostomy to duodenoduodenostomy (1). There were 10 late deaths (range, 3 months to 14 years) attributable to complex cardiac malformations (5), central nervous system bleeding (1), pneumonia (1), anastomotic leak (1), and multisystem organ failure (2). CONCLUSIONS: Late complications occur in 12% of patients with congenital duodenal anomalies, and the associated late mortality rate is 6%, which is low but not negligible. Follow-up of these patients into adulthood is recommended to identify and address these late occurrences.

Abnormalities, Multiple↗