Participants in the Pediatric Surgical Matching Program (PSMP)
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Education, Medical, Continuing↗
Biomedical subjects
D R Cooney
Publications and source records attributed to D R Cooney.
Familial Hirschsprung's disease: report of autosomal dominant and probable recessive X-linked kindreds.
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.
Female↗
Enhanced resection and improved survival in murine neuroblastoma (C1300-NB) after preoperative immunotherapy.
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 immunotherapy may be useful in treatment of advanced human neuroblastoma.
Animals↗
The relationship of class I MHC antigen expression to stage IV-S disease and survival in 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.
Analysis of Variance↗
Postoperative immunotherapy of murine C1300-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.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
The role of pediatric surgery in the medical school curriculum.
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.
Curriculum↗
Immune consequences of nonoperative treatment of splenic trauma in the rat model.
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.
Animals↗
Intramural hematoma of the duodenum.
Intramural duodenal hematoma in children is one of the least common injuries encountered. Because of the rarity of this problem, there has been little conformity of opinion as to the relative merits of operative vs nonoperative management. We reviewed 182 cases of this condition, of which 168 were taken from the English literature and 14 from our hospital. Pancreatitis was the most common associated intra-abdominal lesion (21%), while central nervous system, skeletal, and thoracic injuries (5%) accounted for the major extra-abdominal injuries. Hemophilia, von Willebrand's disease, and idiopathic thrombocytopenic purpura occurred in 7 cases and played a major role in the prognosis. Of these cases, 121 patients were treated surgically and 61 conservatively. The average hospitalization was 14 days for the surgical group and 11 days for the conservative group. There were 18 complications in the surgical group. It appears that most patients with intramural duodenal hematoma would respond well to conservative management. Surgery should be reserved for those cases that remain obstructed over seven to ten days or have evidence of perforation.
Adolescent↗
Long-term pancreatic-biliary diversion in the rat: persistent loss of mucosal enterokinase, with reinduction by delayed oral pancreatic biliary supplementation.
Diversion of pancreatic-biliary (PB) secretions in rats for 23 days led to loss of enterokinase (EK) activity in bypassed segments of the small intestine. Simultaneous oral trypsinogen and bile salt (TB) supplements prevented the loss of EK activity. To study the temporal course of events after PB diversion and to determine if the loss of EK is reversible, PB diversion was performed in rats by surgical transposition of the 4-cm segment of duodenum including the ampulla of Vater to a point 30 cm distal to its original site. Bypassed and control (sham- and nonoperated) rats fed standard rat chow were sacrificed at 10, 23, and 45 days after surgery. One bypassed group was fed standard chow for 23 days and then chow supplemented with TB until sacrifice at 45 days. At sacrifice, the intestines were divided into segment 1 (the bypassed proximal 30 cm) and segment 2 (the 30 cm distal to the bypass). In segment 1, EK disappeared almost completely by 10 days and remained at the same low levels at both 23 and 45 days (p less than 0.05). No significant changes in EK levels were found at any time in segment 2 distal to the bypass. Mucosal disaccharidase activity in segment 1 increased or showed no change. In rats with delayed TB supplementation, EK activity in segment 1 returned almost to control levels at the time of sacrifice. The results confirm the importance of PB secretions in the maintenance of EK activity. The effects of bypass on EK are both enzyme- and site-specific.(ABSTRACT TRUNCATED AT 250 WORDS)
Animals↗
The relative merits of various methods of indirect measurement of intraabdominal pressure as a guide to closure of abdominal wall defects.
Visceral ischemia secondary to increased intraabdominal pressure (IAP) following closure of abdominal wall defects presents a serious postoperative problem. Currently, the method of closure and postoperative management are determined by clinical impressions rather than measurement of IAP. In this study various methods of indirectly measuring IAP were compared in 17 rabbits in which IAP was sequentially increased with an intraabdominal balloon. Vesical and inferior vena caval (IVC) pressures were found to have good statistical correlation with IAP. Other methods tested were gastric, rectal, superior vena caval, femoral and brachial artery, and rectus compartment pressures. All were found to be poor indicators of actual IAP. In nine of the rabbits, radiolabeled microspheres were used to assess cardiac output and visceral blood flow. Renal blood flow was very sensitive to increased IAP with dramatic impairment at IAP above 10 to 15 mmHg. Small intestinal flow was less sensitive and did not become significantly diminished until IAP exceeded 25 to 30 mmHg. Our studies suggest that vesical and IVC pressure monitoring should be used to evaluate IAP in the clinical setting. If IAP is in excess of 10 to 15 mmHg surgical intervention is indicated to prevent the development of renal ischemia.
Abdominal Muscles↗
Presentation of congenital diaphragmatic hernia past the neonatal period.
Congenital diaphragmatic hernias (CDHs) presenting beyond the neonatal period are a rare and unusual problem; they occurred in 11 of 83 children at our institution. Two discrete clinical groups were apparent: (1) younger children, with mainly respiratory symptoms; and (2) older children with gastrointestinal (GI) complaints. Chest roentgenograms suggested CDHs, but GI contrast studies were necessary for confirmation in eight patients. The diagnosis was made preoperatively in ten of 11 children. At operation, no peritoneal sacs were found, the hernial contents were viable in all patients, and malrotation was present in six of 11 patients. None had evidence of pulmonary hypoplasia. Congenital diaphragmatic hernias do present beyond the neonatal age group in a significant number of cases (13% in our series), and a diagnosis of CDH should be considered in any child with persistent GI or respiratory problems and abnormal chest x-ray film findings. The mortality rate in these patients is small, but morbidity may be significant.
Child↗
Present concepts in the treatment of neuroblastoma.
The outlook for the child with neuroblastoma remains dismal. The tumor has been known to undergo spontaneous resolution, and this phenomenon has led to increased interest and research in possible immune mechanisms that may be involved. Treatment often involves the use of surgery, radiation, and chemotherapy, frequently producing a short-term response, but a cure of the disease in an advanced stage can rarely be attributed to any of these treatment modalities. The present staging and treatment method used by the authors is that of the Pediatric Oncology Group (POG) #8104. As far as the immunological therapeutic approach is concerned, promising results have been obtained in animal studies with monoclonal antibodies and immunocellular components. Cumulative data from various pediatric centers have showed that the patient's age strongly influences the prognosis in all stages of the disease.
Child, Preschool↗
Present concepts in the treatment of Wilms' tumor.
Before 1969, 58 patients with Wilms' tumors treated at Children's Hospital of Buffalo had an all-stage survival of 57%. From 1970 to 1983, following the National Wilms' Tumor Study protocols, this survival rate has improved to 79% in the last 47 patients. With continued attention toward early diagnosis, accurate and complete staging, refinement of radiation and chemotherapy protocols, and absolute follow-up of all patients, the relapse-free survival rate will continue to improve.
Child↗
Advances in the treatment of rhabdomyosarcoma.
Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age. Several decades ago it was associated with an 80% mortality rate. Recent advances with combined modality therapy have improved the 5-year survival rate to almost 70%. The focus for the improvement in treatment regimens is now shifting to concerns regarding the preservation of body function and the treatment of far advanced disease. Regardless of age, histology, stage, or site of primary tumor, there has been no group where chemotherapy has not been proven beneficial. The current recommendations used by the Intergroup Rhabdomyosarcoma Study, with regard to chemotherapy, are summarized. Radiation therapy adjunctive to surgery has a useful place in the treatment of rhabdomyosarcoma, but prophylactic nodal irradiation is not recommended. Involved nodes should, however, be treated. Since the potentially adverse side effects of chemotherapy and radiation are accentuated when combined, radiation therapy is no longer recommended in patients with group I rhabdomyosarcoma. Prognostic factors and future considerations from the operative standpoint are presented.
Adolescent↗
Advances in the management of infected urachal cysts.
Persistent urachal remnants are uncommon congenital anomalies. Unless an umbilical fistula exists, infection may be the first indication of this abnormality. Five children received initial treatment for this problem at the Children's Hospital of Buffalo during a 20-year period, 1964 to 1984, and a sixth was seen secondarily. There were four boys and two girls; their ages ranged from 8 months to 9 years. Lower abdominal mass with fever and local tenderness were the most common presenting signs. Ultrasound was the most accurate study, correctly diagnosing the cyst in both patients so examined. Incision and drainage alone was performed in one patient. The other five were managed with antibiotic therapy and complete excision as the primary procedure. Cultures were obtained in five patients and were positive in four, growing Staphylococcus aureus in three and Escherichia coli in one. Significant genitourinary abnormalities were discovered in four of the five patients evaluated. It is concluded that the previously recommended therapy of incision and drainage followed by delayed resection was developed in the preantibiotic era to minimize the mortality from sepsis and the morbidity from recurrence. Our experience indicates that the use of appropriate antibiotics followed promptly by complete cyst excision as a primary procedure is both possible and safe in most cases. Furthermore, the large number of associated genitourinary abnormalities suggests that a complete work-up for these conditions should be performed.
Bacterial Infections↗
Lymphoid cell populations in splenectomized and nonsplenectomized SJL/J mice bearing Hodgkin's disease-like reticulum cell sarcoma.
Using the reticulum cell sarcoma (RCS) of the SJL/J mouse as a model for Hodgkin's disease, the effects of tumor growth and splenectomy on the T-cell and B-cell populations were evaluated. Although splenectomy improved survival and reduced gross observable tumor growth in secondary tumor sites, no effect on T-cell and B-cell populations was detected. We conclude that tumor growth appears to be undetected by the immune system; previously reported immunosuppression in RCS-bearing mice must be due to functional changes and not cell population changes; and splenectomy contributes to survival and suppression of tumor cell growth in secondary sites.
Animals↗
The avoidable excesses in the management of perforated appendicitis in children.
During a five-year period from 1979 to 1985, 100 consecutive children with perforated appendicitis were managed at our institution. These patients were divided into two groups, which were determined by length of illness and physical findings. Group A consisted of 88 children with signs and symptoms of peritonitis from appendiceal perforation. They were treated with fluid resuscitation, antipyretics, and triple antibiotics (ampicillin, gentamicin, clindamycin), and appendectomy within a few hours of hospitalization. Saline lavage was used. Group B was composed of 12 patients with a periappendiceal mass without generalized peritonitis who had symptoms of 6 to 12 days duration. They were treated nonoperatively with triple antibiotics and underwent interval appendectomy 4 to 6 weeks later. The complication rate for Group A was 6.8%. This included three wound infections (3.4%), one intra-abdominal abscess (1.1%), one patient with pneumonia and ileus (1.1%), and a small bowel obstruction (1.1%). These results are equivalent to the lowest complication rate reported in the literature, in which the treatment included transperitoneal drainage, antibiotic lavage, and parenteral antibiotics. Group B patients had a 16.7% complication rate, which included one small bowel obstruction and one recurrent intra-abdominal abscess. Our method of management resulted in the lowest complication rate reported to date in children with perforated appendicitis. Transperitoneal drainage, delayed wound closure, and antibiotic lavage were not used. Subcuticular incisional closure resulted in minimal wound care and excellent cosmetic results. The experience demonstrates that with proper timing of surgery and appropriate use of contemporary antibiotics, the morbidity of perforated appendicitis can be limited and excesses of treatment can be avoided.
Acute Disease↗
Percutaneous central venous catheterization in children: first line choice for venous access.
Percutaneous central venous (CV) catheters using the jugular and subclavian veins have been widely used for hemodynamic monitoring and for venous access in difficult clinical situations. However, peripheral venous cutdowns (PVC) still remain the primary mode of short-term venous access in children. To evaluate percutaneous CV line insertion as a routine procedure, a prospective study of 115 patients (75 CV and 40 PVC) was performed. Ages ranged from 1 day to 17 years; age and weight were similar in both groups. Eighty-three CV lines were attempted, with a success rate of 93%, while 82% of 49 PVC insertions were successful. Complications occurred in 11 (14%) patients with CV access and 31 (78%) with PVC lines. In the latter group, poor flow was a problem in 65%, infiltration in 37.5%, and phlebitis in 27.5%. In the CV group, arterial puncture occurred during insertion in 2 (3%) patients with no adverse sequelae, catheter slippage occurred in 4 (5%), and poor flow in 2 (3%). A large majority (79.2%) of CV lines functioned successfully until no longer needed, as compared with 15% of PVC catheters. The latter were removed before completion of treatment because of poor flow (40%), phlebitis (20%), or catheter problems (10%). Catheter sepsis was documented in only one CV line. Two cutdowns had major local infections. The average catheter longevity was 10.2 days for CV lines, 4.1 days for PVC.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗