Hemolytic uremic syndrome (HUS) is associated with multiple nonrenal manifestations. A unique case is described of a 3-year-old boy who presented with a classic diarrheal prodrome followed by massive necrosis of the biliary tree and common bile duct, pancreas, and the left lobe of his liver. This complication of HUS has not been reported in the English-language literature.

Objective Increasingly powerful nonpowder firearms or air guns are frequently given to children as toys. We undertook the present study to evaluate the injuries caused by these firearms, based on the concern that they are capable of inflicting serious trauma. Design Descriptive, retrospective chart review. Setting Urban level I pediatric trauma center. Participants The study included all children with injuries secondary to air guns who were admitted between July 1988 and March 1995. Main outcome measures Type of weapon, circumstances of injury, anatomic location of injury, injury severity, surgeries performed, morbidity. Interventions None. Results There were 42 admissions with a mean hospital stay of seven days (range 1 to 136 days). The average age was 10 years (range 1 to 23 years) with a median age of 11 years. There were 35 boys and 7 girls. Twenty-nine of the 42 injuries were caused by a family member or friend and five were self-inflicted. The mean injury severity score was 83. While there were no fatalities, 21 children (50%) underwent operative procedures for their injuries. Ten of the injuries were potentially lethal, of which seven were due to the "pump" action air gun. Sixteen patients had serious long-term disability as a result of their injuries. Conclusion Air guns can cause a variety of serious injuries, often requiring operative intervention. The long-term morbidity from some of these injuries is significant Both parents and physicians should be aware that nonpowder guns are not toys, but weapons capable of inflicting serious trauma. The evaluation and treatment of air gun injuries should be similar to that currently used for powder weapon injuries. Recommendations for evaluation and treatment are made.

Objective: To assess the incidence and consequences of small bowel injury (SEI) in children suffering blunt abdominal trauma managed with the intent to treat nonoperatively.Design: Retrospective chart review.Materials and Methods: A total of 168 consecutive hemodynamically stable children admitted to a Level I pediatric trauma center during a 24-month period.Results: Nine of 168 children (5%) sustained SBI: three underwent early (<4 hours) operation for recognized SRI (identified on computed tomographic scan); and six had delayed (36 +/- 16 hours) operation for missed SBI (not identified on computed tomographic scan), Increased temperature and heart rate, or decreased urine output at 24 hours suggested occult SBI, The hospital course was unaltered by delayed diagnosis, Fifty-seven percent of the children (95) suffered intra-abdominal injury; 10% required laparotomy for SBI (9) or solid organ injury (7); 90% (152) were discharged without laparotomy.Conclusions: SBI is uncommon in children suffering blunt abdominal trauma, The diagnosis can be made using clinical and radiographic findings, Limited diagnostic delay does not seem to affect outcome. We conclude that clinical diagnosis of SEI is safe, permits the nonoperative treatment of most blunt abdominal injuries, and reduces the risk of unnecessary laparotomy associated with alternate approaches.

OBJECTIVE:To assess the incidence and consequences of small bowel injury (SBI) in children suffering blunt abdominal trauma managed with the intent to treat nonoperatively.DESIGN:Retrospective chart review.MATERIALS AND METHODS:A total of 168 consecutive hemodynamically stable children admitted to a Level I pediatric trauma center during a 24-month period.RESULTS:Nine of 168 children (5%) sustained SBI: three underwent early (< 4 hours) operation for recognized SBI (identified on computed tomographic scan); and six had delayed (36 +/- 16 hours) operation for missed SBI (not identified on computed tomographic scan). Increased temperature and heart rate, or decreased urine output at 24 hours suggested occult SBI. The hospital course was unaltered by delayed diagnosis. Fifty-seven percent of the children (95) suffered intra-abdominal injury; 10% required laparotomy for SBI (9) or solid organ injury (7); 90% (152) were discharged without laparotomy.CONCLUSIONS:SBI is uncommon in children suffering blunt abdominal trauma. The diagnosis can be made using clinical and radiographic findings. Limited diagnostic delay does not seem to affect outcome. We conclude that clinical diagnosis of SBI is safe, permits the nonoperative treatment of most blunt abdominal injuries, and reduces the risk of unnecessary laparotomy associated with alternate approaches.

C1300 is a murine neuroblastoma that arose spontaneously in an A/JAX mouse, and from which a clone termed TBJ was subsequently derived. C1300 is a slowly growing and poorly metastasizing tumor, whereas TBJ shows early systemic metastasis as well as aggressive local growth. Compared with TBJ cells, C1300 cells are highly immunogenic and are sensitive to natural killer cells and cytotoxic lymphocytes. In vitro, TBJ cells were found to be more rounded and less adherent than C1300 cells. Because the underlying basis for the differences between C1300 and TBJ cells has not been fully elucidated, the authors used high-resolution two-dimensional gel electrophoresis (2-DE) to study comparative aspects of total protein expression by each cell line. Of the approximately 400 individual cellular proteins that could be resolved using this technique, two were found to be reproducibly and uniquely expressed by TBJ cells and not by C1300 cells. Both proteins were anionic (pl 5.0 to 5.2) as assessed by iso-electric focusing and had molecular weights of 76,000 and 82,000 as assessed by sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE). Silver staining of SDS-polyacrylamide gels showed that the levels of 82,000-M(r) protein (p82) were higher than those of the 76,000-M(r) protein (p76). A purification protocol allowing for the isolation of p82 from TBJ cell extracts was developed, which comprised preparative two-dimensional gel electrophoresis followed by reverse-phase high-performance liquid chromatography. Full molecular identification of p82 and p76 eventually may provide new leads in the study of the metastatic or antigenic properties of neuroblastoma.

The management of penetrating neck injuries in adults is controversial, with a trend toward selective neck exploration. These injuries are uncommon in children, and only limited information exists regarding their management. To assess the management of these injuries in the authors' geographic region, they reviewed the records of children with injuries penetrating the platysma muscle who were treated between 1980 and 1994. Forty-six children (aged 2 t0 16 years) suffered a total of 55 penetrating neck injuries. The injuries were classified according to type and location. Fifty-two percent were caused by missiles, 30% by stab wounds, and 18% by dog bites. Fifty-eight percent of injuries were in zone II, 31% in zone I, and only 11% in zone III. The diagnostic workup, including arteriography, esophagography, or endoscopy, was performed preoperatively in 10 patients. Overall, 21 patients had exploration, and the rate of negative explorations was 48%. All cases explored for bleeding or a positive diagnostic workup result were found to have significant injury. On the other hand, all neck explorations performed solely because of injury to zone II were negative. The overall morbidity and mortality rates were 31% and 7%, respectively. A more selective approach, similar to that used for adult patients, emphasizing preoperative diagnostic evaluation, is recommended to decrease the rate of negative neck explorations among children.

In a previous survey of successful candidates (SC) and unsuccessful candidates (USC) to the pediatric surgery matching program (PSMP), significant discriminating factors associated with SC included previous participation in basic science research, number of publications, number of national presentations, and contact with and letters of recommendation from well-known pediatric surgeons. The purpose of this study was to determine the selection criteria used by directors of the pediatric surgery training programs (PSTP) in choosing residents for the PSMP. A two-part survey of the PSMP was completed by directors of the PSTP (n = 24) to identify the factors (n = 31) that most influence their selection process when choosing a resident, and to help clarify questions (n = 11) posed by the candidates during the first survey. In part I, each factor was scored on a modified Likert Scale, from most (1) to least (5) important in evaluating candidates for training positions, and were ranked according to the mean score. Based on our previous survey, but blinded to the PSTP director respondents, these same factors were divided into groups that, in our first survey, did and did not discriminate between SC and USC for the PSMP, and will be referred to as “discriminating factors” (DF, n = 7) and “non-discriminating factors” (NDF, n = 8), respectively. Also, a series of questions dealt with subjective factors of the candidates, not previously analyzed in our original survey, and will be referred to as “program directors' factors” (PDF, n = 16). The three groups (DF, NDF, PDF) were analyzed by the Kruskal-Wallis test and analysis of variance (ANOVA). The five most important factors to the PSTP directors were (1) works well with people (1.24), (2) personal interview (1.33), (3) telephone conversations with other pediatric surgeons (1.4), (4) interviews with other faculty (1.48), and (5) personality (1.5). PDF (mean score, 2.13) were considered most important, followed by DF (2.66) and NDF (3.16). These groups are significantly different by ANOVA (F = 4.16, P = .02) and the Kruskal-Wallis test (H = 6.516, P = .04). Most directors supported mandatory participation in the matching process and were opposed to centralized interviews. Almost all believed that the current system provides accurate information about the candidates, but that qualified candidates often went unmatched. Sixty percent favored an increase in the number of training programs. The PSMP now has been analyzed from both the candidates' and PSTP directors' points of view. The results of this survey of the PSMP by the PSTP directors confirm our previous survey's results with respect to objective factors that distinguish between SC and USC in the PSMP. However, subjective PDF were considered the most important factors in evaluating a candidate; this emphasizes the personal intangible characteristics of the candidates and assumes stellar academic and technical abilities.

Hirthler, M. A.; King, D.; Caniano, D.; Boles, T.; Cooney, D. R.; Teich, S. Author Information

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and pulmonary hypertension. We hypothesize that a surfactant deficiency may in part be contributing to the pathophysiology of CDH. This study documents the functional, quantitative, and qualitative aspects of the surfactant status of the alveolar air-liquid interface and the type II pneumocyte in the fetal lamb CDH model. Ten lamb fetuses (gestational age, 80 days) had a CDH created via a left thoracotomy and then were allowed to continue in utero development until term. Three litter mates and three nonoperated time-dated fetuses served as controls. At term, pressure-volume curves were performed to measure pulmonary compliance and total lung capacity. Alveolar lavage was then performed to measure the quantitative and the qualitative aspects of pulmonary surfactant. Finally, isolation of type II pneumocytes allowed quantification of phospholipid synthesis. When compared with controls (N = 6), the CDH lambs (N = 5) had significantly smaller lungs (P = .009), decreased total lung capacity (P < .001) and compliance (P < .001), reduced total lavaged phospholipids (P = .006), and decreased percent phosphatidylcholine (P = .02). CDH lambs also had increased total lavaged proteins (P = .05) and higher minimum dynamic surface tension (P < .001). A surfactant deficiency may be contributing to the pathophysiology of CDH. Surfactant replacement therapy in premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Exogenous surfactant may also benefit infants with CDH.

Exogenous surfactant therapy (EST) in surfactantdeficient premature infants has been shown to improve lung compliance, decrease morbidity, and improve survival. Reports have demonstrated that newborns with congenital diaphragmatic hernia (CDH) have lung compliance, pressure-volume curves, and hyaline membrane formation resembling those changes seen in surfactant deficient premature newborns. We hypothesize that EST may also benefit infants with CDH. All high risk cases of prenatally diagnosed CDH at Children's Hospital of Buffalo from November 1988 to February 1991 were prospectively evaluated for EST. In those families who chose to participate, the surfactant preparation, Infasurf (100 mg/kg), was instilled into the newborn's lungs prior to the first breath. The remainder of the perinatal, neonatal, and surgical care was performed in a routine manner. Three high-risk prenatally diagnosed newborns with left CDH were treated with EST. All showed signs of decreased pulmonary compliance, but could still be adequately oxygenated and ventilated. Surgical correction was performed after stabilization and all required patch closures. Two of the three infants suffered no life-threatening episodes of pulmonary hypertension and all survived. These infants had many known indicators for poor outcome in CDH with an expected survival of less than 20%. We believe that EST in these neonates with CDH contributed to their survival with minimum morbidity. These results suggest that surfactant replacement for the high-risk neonate with CDH warrants further consideration and a randomized clinical trial is being planned.

Only one third of the applicants for training in pediatric surgery obtain a position through the pediatric surgery matching program. In order to identify factors that contribute to a successful outcome, we conducted a retrospective survey of all participants in the matching process for positions during the years 1983 to 1991. This survey was designed to identify characteristics associated with success in the match through comparison of successful and unsuccessful applicants. Significant factors associated with a successful match included: a greater incidence of residency-supported research (P = .012) with a greater number of publications (P = .003) and national presentations (P = .014), specifically at the annual meetings of the American Pediatric Surgical Association (P = .05) and the American Academy of Pediatrics (P = .05). In addition, successful candidates had more contact with (P = .004) and letters of recommendation from (P = .015) well-known pediatric surgeons involved in the general surgical residency program. This information should be invaluable to those counseling medical students and residents interested in a career in pediatric surgery.

We report a patient with congenital neutropenia or Kostmann's Syndrome who suffered many complications after presenting with Clostridium septicum enterocolitis, including absence of wound healing. Because of several reports of the use of granulocyte colony-stimulating factor (G-CSF) in patients with various complications of neutropenia, we treated this patient with recombinant human (rh) G-CSF. We found that once rhG-CSF restored neutrophil counts to normal, progressive wound healing followed. Thus, rhG-CSF therapy may be useful in treating neutropenic patients with wound complications.

Multifactorial sex-modified inheritance has been proposed as the model of transmission in familial Hirschsprung's disease (HD). A review of two separate kindreds suggests that aganglionosis may be inherited as an X-linked recessive or an autosomal dominant trait. Chromosomal anomalies and other syndromes, including G6PD deficiency, may occur with familial HD. Recurrence risk counseling for family members depends on accurate pedigree analysis and a comprehensive understanding of the genetic factors involved.

Advanced neuroblastoma treated with standard chemotherapy has a poor prognosis. Combination immunotherapy for murine neuroblastoma with retinyl palmitate, low-dose cyclophosphamide, and interleukin-2 resulted in increased survival, impaired tumor growth, easier surgical resection, and increased class I expression or tumor cells. Preoperative immunotherpay may be useful in treatment of advanced human neuroblastoma.

Cultured human neuroblastoma cells express low levels of class I (MHC) surface antigen. In order to determine if this low expression is representative of the clinical tumor, this study investigates class I expression in archival human neuroblastoma. Whereas stages I to IV neuroblastoma expressed low levels of class I antigen, stage IV-S tumor cells expressed normal levels, similar to control tissues. Expression of class I antigen in tumors from survivors of stage III neuroblastoma was significantly greater than in tumors from nonsurvivors. Tumors comprised predominantly of ganglion cells expressed significantly more class I antigen than neuroblasts. These data suggest that class I MHC expression may play a role in the natural history of human neuroblastoma.

Low-dose cyclophosphamide (CY) is an immunomodulating agent that down-regulates T suppressor cell function. This study investigates postoperative immunotherapy with CY as an alternate treatment for advanced immunogenic tumors such as neuroblastoma that typically respond poorly to traditional high-dose chemotherapy. A/J mice with 1.5-cm subcutaneous C1300-neuroblastoma (C1300-NB) tumors were divided into the following treatment groups: I, untreated (n = 14); II, 85% tumor resection (n = 18); III, sham-operated (n = 18); IV, multiple-dose CY (n = 6); V, 85% resection and single-dose CY (n = 14); VI, 85% resection and multiple-dose CY (n = 14). CY (100 mg/kg, intraperitoneally) was given initially 24 hours post-operatively to groups IV, V, and VI. Groups IV and VI also received weekly maintenance doses of 25 mg/kg CY. Results showed significantly increased survival (log-rank test) in CY-treated groups (IV, V, VI) compared with control groups (I,II,III). Cures were observed only in groups receiving partial resection plus CY (V, 7%; VI, 29%). Although surgical debulking of tumor alone (II) did not enhance survival, the procedure normalized depressed total lymphocyte counts and the subpopulation of Lyt 2,3+ (T suppressor/cytolytic cells) in the immediate postoperative period during which immunotherapy with CY was instigated. This may have contributed to the success of CY immunotherapy. To characterize the tumor-host immune interaction, additional studies were performed. Results showed the following. (1) Mice cured by debulking plus CY (from groups V and VI) could not be successfully reimplanted with C1300-NB, demonstrating immunologic mediation by CY. (2) Injection of antithymocyte serum abrogated the therapeutic effects of CY in tumor-bearing mice, indicating that CY action is T cell-mediated. (3) T cell Concanavalin-A blastogenesis assay demonstrated suppression of T cell stimulation in tumor-bearing mice. This tumor-associated suppression was eradicated by CY treatment (100 mg/kg) of tumor-bearing mice. (4) Treatment of tumor-bearing mice with intravenous anti-IJK serum significantly delayed tumor growth (P = .002), demonstrating a role of IJK cells in C1300-NB growth. These IJK cells are part of the T suppressor network. (5) An increased polymorphonuclear cell count in the peripheral blood was a marker for the presence of tumor tissue, even before the gross tumor was noted. Because this evidence suggests that tumor-associated T suppressor populations contribute to experimental neuroblastoma growth, a trial of treatment of neuroblastoma patients with low-dose CY to inactivate these populations and support host antitumor mechanisms may be indicated, especially in the face of treatment failures with high-dose CY.

To investigate the immunologic consequences of non-operative management of splenic injury, three parameters were studied: survival following pneumococcal sepsis, clearance of blood-borne bacteria, following Hemophilus influenzae challenge, and antibody response to type III pneumococcal capsular polysaccharide. Two hundred twenty-five Sprague-Dawley rats were divided into three groups and subjected either to a splenectomy, a sham operation, or standard blunt trauma. A significant increase in mortality was noted in the splenectomized group as compared with both the traumatized and control groups when challenged with Streptococcus pneumoniae. In both the control and trauma groups, H influenzae cleared significantly within 18 hours. Blood-borne bacteria persisted at the same level for 72 hours in the splenectomized animals. Four and 11 days later, the antibody level in both traumatized and control groups was higher than in the splenectomized subjects (P less than .001). There was no difference in the serum antibody level between the control and trauma groups at four days. However, at 11 days the trauma group showed a significant decrease in the antibody level (P less than .05). It can be concluded that following spontaneously-healing splenic trauma in the rat model, survival, bacterial clearance, and antibody response were all superior to that observed in the splenectomized subjects. In addition, the healed splenic disruption did not impair clearance of blood-borne encapsulated bacteria.

In most medical schools, exposure to pediatric surgery is presented as a subspecialty elective. We have offered it as an integral part of the surgical clerkship for 10 years in the belief that it provides an excellent educational environment. To confirm this concept, the quizzes (Q), final examinations (FE), and grades of students assigned to the pediatric surgical service were prospectively studied. All students (N = 139) in the surgical clerkship entered the study. Thirty-two students were randomly selected and assigned to the surgical service of a major pediatric hospital (P-Surg) for 50% of their clerkship. The other students (N = 107) were assigned to a variety of adult surgical services (G-Surg) and served as the control group. All students attended the same seminars, used the same educational materials, were examined with the same test items, and were evaluated by the same oral examiners. Test items were electronically scored and the database was analyzed on an IBM computer. The statistical analysis was performed using a Student's t test and chi 2 analysis. There was no significant difference in the demonstrated cognitive performance and grades awarded to the two groups of students. We conclude that a pediatric surgical service provides an atmosphere that is educationally comparable to the adult general surgical service.