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Biomedical subjects

R S Sawin

Publications and source records attributed to R S Sawin.

12 recordsLinked to original sources

Preresection chemotherapy improves survival for children with Askin tumors.

OBJECTIVE: To test whether patients with Askin tumor treated with aggressive neoadjuvant chemotherapy have a better clinical outcome. DESIGN: Retrospective case series. SETTING: Pediatric referral center. PATIENTS: All children diagnosed with malignant small-cell tumors of the chest wall (Askin tumor) and treated from 1975 to September 1987 (phase 1, n = 6) and from September 1987 to the present (phase 2, n = 9). MAIN OUTCOME MEASURES: Survival as a function of extent of disease and response to therapy as measured by tumor volume, survival, and recurrence. RESULTS: All phase 2 patients had significant reduction of tumor volume and improved survival by Kaplan-Meier estimates compared with phase 1 patients. No phase 1 patients are still alive. CONCLUSION: Patients with Askin tumor treated with aggressive preresection chemotherapy have smaller tumors to resect and improved survival.

Adolescent

Surgical implications of necrotizing fasciitis in children with chickenpox.

Varicella (chickenpox) affects approximately 90,000 children each year. Although most cases resolve, some develop necrotizing soft tissue infections secondary to group A streptococcus and staphylococcus. Delay in diagnosis is common. At the time of initial presentation, the need for surgical intervention is not always clear. The authors conducted a retrospective review of 30 patients with varicella (seen from December 1993 to June 1995) for whom there was clinical concern for necrotizing soft tissue infection. Various parameters were examined, including tachycardia, band count, temperature, and clinical symptoms, to differentiate the children who required surgery from those who did not. Of the 30, 22 underwent surgery. Eighteen had necrotizing fasciitis and required debridement, and four had abscesses that were incised and drained. Eight patients had simple cellulitis and did not require operation. Group A streptococcus was the most common organism cultured. All patients were treated with appropriate antibiotics. Twenty of the 22 surgical patients had elevated band count (> or = 5%), 21 had tachycardia, and 18 were febrile at the time of presentation (> 4 days after the onset of chickenpox). Although all patients with necrotizing fasciitis had tachycardia, this sign was a less specific indicator for surgery than was increased band count. Severe pain, erythemia, and induration was the most common signs/symptoms in the surgical patients. The survival rate for these 30 patients was 100%, and there was little long-term morbidity. The authors recommend immediate surgical intervention for children with chickenpox who present more than 2 or 3 days after the onset of the viral illness with symptoms that include fever, tachycardia, and an elevated band count in association with an erythematous, indurated, painful lesion. With this sign/symptom complex, the presumptive diagnosis must be necrotizing fasciitis until proven otherwise. If the patient has suspicious symptoms or if these symptoms are associated with tachycardia or an elevated band count, the patient warrants admission, institution of intravenous fluids, nafcillin, clindamycin, and close observation over several hours. If the symptoms progress over the next few hours or if the tachycardia persists despite rehydration and antibiotics, the patient should be taken to the operating room for exploration. The authors strongly endorse such exploration despite the risk of a negative operation, because the morbidity and mortality associated with delayed surgical treatment are potentially significant. With prompt aggressive surgical and medical treatment, a good outcome can be anticipated for these patients.

Anti-Bacterial Agents

Does posterior sagittal anorectoplasty in patients with high imperforate anus provide superior fecal continence?

The posterior sagittal anorectoplasty (PSARP) has become the primary surgical procedure for patients with high imperforate anus. Very few careful long-term follow-up studies have established the superiority of the PSARP procedure over other surgical repair techniques. The authors' goal was to evaluate the operation and to identify the factors associated with improved continence. The medical records, operative reports, and radiographs of 53 patients (46 male, 7 female) who underwent PSARP for high imperforate anus at Children's Hospital and Medical Center (CHMC) between 1982 and 1990 were reviewed retrospectively. Subjective follow-up data were collected by telephone questionnaire, assessing habits indicative of stool continence, and a "fecal continence score" (FCS) was calculated for each patient. A prospective, 7-day diary assessing similar patterns of fecal continence was completed by each patient's family. The mean age of the patients studied was 8.0 years, and the mean follow-up period was 6.8 years. The telephone questionnaire was completed for 48 patients (94%). Toilet training for bowel continence was successful in 20 patients (42%) and occasionally successful in another 20 patients (42%), but 8 patients (16%) had no awareness of impending stool. Forty-five (94%) were physically active, but 16 (33%) reported social problems related to offending odor. The mean fecal continence score for all patients was 3.0 +/- 1.4 (5=excellent, completely continent), which was nearly identical to published scores for the other types of surgical repair. The FCS did not improve with age. The parents' responses to the telephone questionnaire matched the results obtained from the prospectively collected continence diary data. The authors' results for PSARP in patients with high imperforate anus do not differ substantially from those achieved by other repair techniques. Previously cited prognostic factors such as fistula anatomy and sacral anomalies did not appear to alter the functional results in the authors' series. Aggressive postoperative bowel management should be anticipated in all patients who have high imperforate anus and may benefit those who otherwise would achieve less satisfactory continence.

Adolescent

Laparoscopic Ladd's procedure and assessment of malrotation.

Upper gastrointestinal series are frequently obtained on children with abdominal pain and vomiting. If the ligament of Treitz (LOT) is in an equivocal position and not to the left of the spine at L1 or L2 it is important to determine the position and fixation of the cecum to assess the breadth of the mesenteric pedicle. Radiographic studies may not accurately accomplish this because of the changing position of the cecum. Laparoscopy can he used to determine the position of LOT and whether the cecum is fixed in the right lower quadrant. If the patient is judged to be at risk for volvulus (i.e., a shortened mesenteric pedicle) a Ladd's procedure can be accomplished laparoscopically with good long-term results. The use of the laparoscope is not advocated when acute volvulus is suspected.

Cecum

Secretion of gastrin-releasing peptide by retroperitoneal tumors.

PURPOSE: Gastrin-releasing peptide (GRP) is a neuropeptide with growth factor activity in vitro for a variety of tumors including neuroblastoma. If GRP is secreted by neuroblastomas, its detection in serum might be an excellent way to both diagnose and monitor this tumor in patients. METHODS: Small portions of resected tumor specimens were maintained in tissue culture as tumor explants for 24 hours. The tumors included: 3 ganglioneuromas, 1 neuroblastoma, 1 primitive neuroectodermal tumor, 1 Wilms' tumor, 1 rhabdoid tumor, and 1 benign brachial plexus tumor. Control flasks were maintained simultaneously under identical conditions. After 24 hours of incubation, the tumor-conditioned media and the control media were assayed in duplicate for [GRP] using a radioimmunoassay. RESULTS: All the conditioned media from the benign tumors contained < 25 pg/mL net GRP, whereas all the malignant tumor-conditioned media contained > or = 45 pg/mL (P = 0.003). CONCLUSIONS: These data suggest that GRP is secreted by pediatric retroperitoneal tumors and that the amount secreted varies directly with the degree of malignancy of the tumor. This study suggests that GRP may be a candidate tumor marker for pediatric retroperitoneal tumors.

Biomarkers, Tumor

Early recognition of neonatal abdominal wall necrotizing fasciitis.

Necrotizing fasciitis (NF) of the abdominal wall occurring in newborns is associated with a 50% mortality rate. Improved survival requires early diagnosis followed by aggressive surgical débridement. During a 10-year period, we treated 7 infants who developed NF. During the same period, 32 infants were admitted with omphalitis that did not progress to NF. The patients with omphalitis and those with NF were compared. Tachycardia, abnormal white blood cell counts, induration, and violaceous skin discoloration were seen exclusively in the NF patients. Polymicrobial infections were documented in 28% of the omphalitis patients and 86% of the NF patients. All omphalitis patients survived, whereas 5 of 7 (71%) NF patients died. Adjuvant hyperbaric oxygen therapy was used for 4 infants with NF, 2 of whom survived (50%). NF is a highly morbid disease, that can be distinguished from other infant abdominal wall infections by the skin changes, white blood cell counts, heart rate, and microbiologic results. Prompt diagnosis of NF improves survival when combined with aggressive surgical débridement.

Abdominal Muscles

Repair of a laryngotracheoesophageal cleft in an infant by means of extracorporeal membrane oxygenation.

Few survivors have been reported following attempted repair of laryngotracheoesophageal clefts (LTECs). The major challenge is maintaining oxygenation, both during the surgical repair and during the postoperative period of healing. We report a neonate with an LTEC extending to the carina whose successful repair was facilitated by extracorporeal membrane oxygenation (ECMO) begun intraoperatively and continued postoperatively for 11 days. The intraoperative surgical exposure of the defect was excellent. Postoperative trauma to the fresh tracheal repair from ventilatory pressures and endotracheal tube motion was eliminated through the use of ECMO. The patient was discharged without a tracheotomy and with a normal voice, cry, and swallow. According to this result, the use of ECMO may represent a significant advance in facilitating the correction of major laryngotracheoesophageal anomalies. The rationale, advantages, disadvantages, and potential pitfalls of this approach are presented, as well as preoperative and postoperative documentation of our results.

Congenital Abnormalities

Appendiceal US scans: histologic correlation.

High-resolution in vitro ultrasonography (US) of 20 surgical appendiceal specimens was performed to compare appearances of appendiceal tissue at US with corresponding histologic features. With an articulated-arm system and micropositioner, precise spatial correlation was achieved. As elsewhere in the gastrointestinal tract, five distinct echo layers were observed. Normal and inflamed specimens demonstrated these layers, but the architecture became disorganized and indistinct in cases of appendicitis. Three measurements were made for each specimen: (a) overall cross-sectional diameter, including the lumen, (b) thickness of the submucosal echo layer, and (c) the combined thickness of both walls, excluding the lumen. For the inflamed specimens, a substantial increase in the thickness of the summed wall measurements was found. Wall US appearance alone may be misleading in differentiation of normal and abnormal appendices.

Adolescent

Necrotizing enterocolitis in term neonates.

Necrotizing enterocolitis (NEC) is usually a disease of premature infants, but occasionally it affects the term neonate. A 5-year review of NEC at Children's Hospital and Medical Center identified the unique features of this disease in the term neonate. Eighty-one patients with NEC were treated between January 1984 and May 1989. Ten full-term neonates with gestational age greater than 38 weeks were identified for study. Charts were reviewed for recognized risk factors, clinical course, surgical intervention, and outcome. Ninety percent had a birth weight greater than or equal to 2.7 kg, and all were above 2.1 kg. NEC developed early in this group, with onset of disease in the first 48 hours of life in 50% of the group and within the first 4 days of life in 90%. The recognized risk factors of asphyxia, hypoglycemia, polycythemia, and respiratory distress were absent in 60%. Seven of 10 patients required exploratory laparotomy, whereas 3 of 10 required only medical treatment. Indications for operation were perforation in three patients, peritonitis in three patients, and mass in one patient. All patients requiring operations had severe colonic disease, with perforation of the colon in five of seven and full-thickness necrosis without perforation in two of seven. Two patients required total abdominal colectomy. Only one patient with perforated meconium ileus and associated NEC had small bowel involvement. This patient was the only mortality of the group. Subsequent intestinal continuity was restored in all surviving patients with no late complications. Two patients required resection of additional NEC strictures prior to reanastomosis. Of the three medically treated patients, none required subsequent operation for colonic stricture. Our experience indicates that the presentation, clinical course, and operative findings in full-term neonates with NEC differ from those encountered in the premature infant with NEC.

Birth Weight

Staged repair of interrupted aortic arch with ventricular septal defect compared with primary repair in infancy.

Interrupted aortic arch is a poor prognosis cardiac anomaly with nearly 100 percent mortality if not recognized and treated early. The associated intracardiac lesions often lead to death if only the arch defect is repaired. Several recent reports have described patients with interrupted aortic arch who were treated as infants by primary repair of the arch defect with simultaneous repair of the intracardiac lesions. The improved survival data from these series have been attributed to the simultaneous repair of both lesions. We report herein on nine patients with both interrupted aortic arch and ventricular septal defect seen at Children's Hospital and Medical Center in Seattle from 1979 to 1987. Three patients had partial expression of DiGeorge's syndrome. All patients underwent primary repair of the interrupted aortic arch with concomitant pulmonary artery banding during infancy (mean age 18 days, range 2 days to 4 months). Operative mortality was 11 percent (1 of 9 patients). Eight patients had eventual repair of the ventricular septal defect (mean age 18 months, range 6 to 29 months) with one death occurring at 5 months postoperatively (12 percent mortality). The overall mortality of these nine patients was 22 percent. Staged repair of interrupted aortic arch with associated ventricular septal defect can be performed with results comparable to simultaneous primary repair in infancy. The improved survival from either approach is more likely to be attributable to improved perioperative stabilization, particularly the use of prostaglandin E.

Age Factors

Epidemiologic relationships of the bovine population and human leukemia in Iowa.

Data from the Third National Cancer Survey and data collected under the auspices of the National Cancer Institute's Surveillance, Epidemiologic and End Results (SEER) Program were used to perform a descriptive epidemiologic study of leukemia in Iowa. Data were also collected on livestock population patterns in the state, and a survey was performed to locate cases of lymphosarcoma within the cattle population. Ecologic relationships between human leukemia, livestock populations and bovine lymphosarcoma were investigated. Iowa has higher rates than the national average for human leukemias. The lymphoid leukemias make up the majority of the excess leukemias. Excessive acute lymphoid leukemia (ALL) is seen in the ages under 20 years and over 60 years. The excess ALL is in males living in rural counties. There is no sex difference for chronic lymphoid leukemia (CLL), but there is a slight urban-rural differential. There is a high positive correlation between ALL in males and cattle density. This relationship is greater for daily cattle than for beef cattle. There is an additional positive relationship between counties with excessive ALL and the presence of dairy herds affected with bovine lymphosarcoma.

Adolescent