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Biomedical subjects

J L Grosfeld

Publications and source records attributed to J L Grosfeld.

At least 127 records · Page 7Linked to original sources
PubMed1990

Delayed onset of intestinal obstruction in children after unrecognized seat belt injury.

The increased use of child safety seats and seat belt restraints has significantly reduced the incidence of severe head injuries associated with motor vehicular accidents. However, an increase in the number of both acutely recognized intestinal perforations and delayed obstructions due to ischemic strictures has been noted. This report describes two children with delayed onset of intestinal obstruction related to the "seat belt syndrome" who presented with bilious emesis 3 to 6 weeks following an unrecognized lap belt injury. At laparotomy, a volvulus around an omental band adherent to a resolving traumatic mesenteric hematoma was the basis of the obstruction in both cases. The volvulus resulted in a stricture in each instance that required resection and end-to-end anastomosis. The diagnosis of posttraumatic intestinal obstruction should be suspected in children who develop nausea and bilious emesis following motor vehicular accidents in which they were wearing lap belts.

Abdominal Injuries↗
PubMed1990

Acquired aganglionosis: a rare occurrence following pull-through procedures for Hirschsprung's disease.

Hirschsprung's disease (HD) is a neurogenic form of intestinal obstruction characterized by a congenital absence of ganglion cells in the submucosal and myenteric plexuses. Acquired aganglionosis (AAG) is a rare condition that may occur following various pull-through procedures for HD. This report describes five boys with acquired aganglionosis. In all cases, the presence of normal ganglion cells was confirmed on review of biopsies of the pull-through segments at the initial operation. The original pull-through procedure included Soave (2), Duhamel (2), and Swenson (1) operations. Three procedures were initially performed at other institutions. Recurrent symptoms including abdominal distention, obstipation, enterocolitis, and failure to thrive developed 7, 11, 12, 18, and 30 months postoperatively (mean, 15.6 months). The diagnosis of AAG was delayed 1.5 to 9 years after the onset of recurrent symptoms. Full column barium enema studies revealed a transition zone or narrow area in the rectosigmoid or descending colon in four children. Repeat full thickness rectal biopsies at 3.0 cm above the anal verge in the pull-through segment confirmed the absence of ganglion cells in each case. Two children (post Swenson and Duhamel) were successfully revised with a Swenson procedure. Two additional children (post Soave and Duhamel) were successfully treated with extended posterior anomyomectomy procedures. The remaining boy now has a preliminary colostomy and is awaiting a second procedure. Vascular compromise of the distal bowel segment at the time of the initial pull-through procedure may contribute to the selective loss of ganglion cells postoperatively as neural tissues are most sensitive to hypoxia.(ABSTRACT TRUNCATED AT 250 WORDS)

Colon↗
PubMed1990

Peritoneal dialysis in the first 60 days of life.

This report describes a 7-year experience with acute peritoneal dialysis in 31 neonates and infants less than 60 days of age. There were 20 boys and 11 girls, ages 3 to 60 days. Tenckhoff catheters of modified length were placed in the newborn intensive care unit (ICU), pediatric ICU, or surgery suites, and hourly exchanges (20 cc/kg) were started immediately postoperatively. Diagnoses included congenital metabolic disorders (11), acute tubular necrosis (6), postcardiopulmonary bypass with renal failure (5), renal cortical necrosis (5), obstructive uropathy (2), renal agenesis (1), and bilateral renal dysplasia (1). Complications included: peritonitis (4), bowel perforation (1), exit site infection (3), leaking dialysate (4), catheter obstruction (2), inguinal hernias (3), umbilical hernia (1), and retroperitoneal hemorrhage (1). There were 19 deaths (61.3%) from 1 to 90 days postinsertion in this high risk group. The (1), and post liver transplant (1). Effective dialysis (lowering of blood urea nitrogen (BUN) or ammonia, correction of acidosis, decrease in fluid overload) was possible in all cases. Five of the 12 survivors remain on chronic dialysis awaiting renal transplantation. Peritoneal dialysis is effective in the newborn period in the management of metabolic disturbances as well as renal failure. Morbidity and mortality (61.3%) is related to the near-morbid condition of the baby at the time of insertion and the severity of the complex underlying diagnosis often associated with multiorgan failure.

Acute Kidney Injury↗
PubMed1990

Hepatic resection for disseminated germ cell carcinoma.

Improvements in operative technique and perioperative management have expanded the application of hepatic resection for metastatic cancer. Although a policy of aggressive surgical resection of residual pulmonary and retroperitoneal disease following chemotherapy and normalization of serum tumor markers has been adopted for disseminated germ cell carcinoma, resection of residual hepatic disease in these cases has not been addressed. This report concerns a series of prospectively randomized patients who received systemic cisplatin-based chemotherapy for testis cancer during the past 13 years. Twenty-eight patients underwent resection of residual hepatic disease after serologic remission. Most (23 of 28 patients) of these procedures were performed concomitantly with other cytoreductive procedures. There were no operative deaths, although 28% of the patients developed complications. The 2-year survival rate was 54%, with an average follow-up of 34 months. Patients were stratified into three groups based on the most aggressive histology noted in the resected specimen. Survival is predicted by this histologic classification system. Hepatic resection can be performed safely and is an important component in the treatment of disseminated testicular carcinoma.

Adolescent↗
PubMed1990

The diagnosis and definition of hepatic malignancies by use of arterial enhanced computerized tomographic scanning.

Axial computerized tomography is a useful tool in the evaluation of either primary or metastatic hepatic neoplasms. An adjunct to this technique is visceral arterial enhanced computerized tomography (AECT). To determine the effectiveness of this modality, bolus intravenous enhanced computerized tomography scans and AECT were compared and correlated to operative findings. Fifty-four consecutive patients were evaluated by AECT and bolus intravenous enhanced computerized tomography over a 30-month period (May 1986 to August 1989) for suspected primary or metastatic hepatic malignancies. Forty-four patients (81%) had hepatic lesions. Fifty-two percent (23 of 44 patients) of the metastatic tumors were from colonic or rectal primary lesions, and 20% were hepatocellular primary lesions. The remainder of the lesions were metastases from a variety of primary lesions. When studies were compared, 34% of the patients (15 of 44 patients) differed in either the location or total number of lesions noted. The lesions of three of the 15 patients (20%) were determined unresectable on the basis of AECT. Of the remaining patients, planned resections were revised in seven patients to either lesser or greater procedures. The number of lesions found at laparotomy equaled the number found by AECT in all but two cases. AECT caused no complications. AECT improved our ability to identify and localize primary and metastatic lesions of the liver. This technique offers the advantage of preoperative definition of the hepatic arterial and portal venous anatomy.

Adenoma, Islet Cell↗
PubMed1990

Anomalies of intestinal rotation in childhood: analysis of 447 cases.

This report concerns 447 infants and children with anomalies of rotation and fixation. Patients were placed in four groups based on initial symptoms. Group A involved 18 patients with acute midgut volvulus. At laparotomy, midgut volvulus was noted and reduction of midgut volvulus and a Ladd procedure were performed in 10 cases and resection was required in 8. There were five deaths (28%). Group B included 54 children with chronic symptoms of intermittent volvulus or duodenal obstruction. Group C involved 44 cases of malrotation observed during exploration for other disorders. Patients in groups B and C underwent a Ladd procedure and appendectomy. There were five unrelated deaths. Group D included 331 neonates with malrotation caused by either diaphragmatic hernia (n = 111) or abdominal wall defects (n = 220). A Ladd procedure was performed on 48 patients with abdominal wall defects and 29 surviving children with diaphragmatic hernia. Only 2 of 172 (1.2%) patients with abdominal wall defects and 1 of 34 (2.9%) patients with diaphragmatic hernia not treated for malrotation had midgut volvulus. Midgut volvulus is more common in infants and is associated with a high mortality rate (28%). Patients with malrotation and chronic obstructive symptoms or those observed during other elective procedures should undergo a Ladd procedure because of the risk of midgut volvulus. The risk of midgut volvulus is low in patients with abdominal wall defects and, probably as a result of adhesions from previous neonatal operations.

Appendectomy↗
PubMed1989

Integration of nutrition support into oncologic treatment protocols for high and low nutritional risk children with Wilms' tumor. A prospective randomized study.

Benefits and risks of nutrition support were evaluated in 31 malnourished children with newly diagnosed Wilms' tumor managed according to the third National Wilms' Tumor Study protocol. Patients were classified at diagnosis as being at high nutritional risk (HNR, n = 19) or low nutritional risk (LNR, n = 12). Ten HNR patients were randomized to central parenteral nutrition (CPN) and nine HNR patients were randomized to peripheral parenteral nutrition (PPN) plus enteral nutrition (EN) for 4 weeks of initial intense treatment and EN (nutritional counseling, oral foods and supplements) thereafter. Thirteen HNR patients (seven CPN, six PPN) completed the protocol. Twelve LNR patients received EN; 11 Stage I malnourished patients were randomized to 10 or 26 weeks of chemotherapy. Dietary, anthropometric, and biochemical data were determined for HNR patients at weeks 0-4, 6, 13, 19, and 26 and for LNR patients at weeks 1, 2, 5, and 26. In HNR patients, adequate parenteral nutrition support reversed protein energy malnutrition (PEM), and prevented chemotherapy and radiotherapy delays due to granulocytopenia. CPN was superior to PPN in reversing PEM: energy intake, weight gain, and retinol binding protein were higher (P less than 0.05). LNR patients lost weight and fat reserves in the first 2 weeks of treatment; depletion persisted at week 5, and 25% had chemotherapy delays. Thereafter, EN reversed PEM in patients with both chemotherapy regimens. These data suggest that CPN is preferable during initial intense treatment for HNR patients, and that, although EN is ineffective in preventing depletion and treatment delays in the first 5 weeks of treatment for LNR patients, it is effective thereafter.

Abdomen↗
PubMed1989

Effectiveness of two methods of parenteral nutrition support in improving muscle mass of children with neuroblastoma or Wilms' tumor. A randomized study.

The short-term and long-term effectiveness of central parenteral nutrition (CPN) versus peripheral parenteral nutrition (PPN) in improving muscle mass (arm muscle area [AMA]) was evaluated for 24 malnourished children with newly diagnosed Stage IV neuroblastoma (n = 14) or Stages II-V Wilms' tumor (n = 10). Patients were randomized to either CPN or PPN plus enteral nutrition (EN: intense nutrition counseling, oral foods, and supplements) for 4 weeks followed by EN until week 10. Oncologic treatment was similar for each tumor type. Dietary, anthropometric, and biochemical measurements were obtained at weeks 0, 4, and 10. During weeks 1 through 4, energy (CPN: means 100 +/- 4; PPN: means 96 +/- 4% of healthy children) and protein (CPN: means 2.5 +/- 0.1; PPN means 2.7 +/- 0.2 g/kg) intakes of the two groups did not differ. The AMA increased (P less than 0.05) with 4 weeks of CPN but not with PPN; changes thereafter with EN were not significant. Weight (P less than 0.05) and triceps skinfolds (P less than 0.01) increased with 4 weeks of PN in both groups and decreased with EN thereafter (P less than 0.01) but were higher at week 10 than diagnosis. Increases in albumin in both groups reached significance at week 10 (P less than 0.05). These data show that CPN improves AMA in malnourished children with neuroblastoma or Wilms' tumor when energy and protein intakes are adequate. The AMA gains can be maintained thereafter with EN.

Anthropometry↗
PubMed1989

Changing patterns of treatment and survival in neonates with meconium ileus.

This report describes 51 neonates with meconium ileus and emphasizes a changing pattern of treatment and improved survival. Twenty-four neonates had uncomplicated meconium ileus due to inspissated meconium obstructing the distal ileum. Twenty-seven neonates had 41 complications of meconium ileus including volvulus (18), bowel atresia (13), perforation (5), and giant cystic meconium peritonitis (5). Nine patients with uncomplicated cases responded to nonoperative clearing of meconium using a meglumine diatrizoate (Gastrografin) enema. Six of 7 patients with enema failures underwent laparotomy, purse-string enterotomy, and intraluminal irrigation. The remaining 9 patients with uncomplicated meconium ileus had resection and enterostomy. Complicated cases were managed by resection and anastomosis (13) or enterostomy (14). Survival at 1 year was 92% in patients with uncomplicated meconium ileus and 85% for those with complicated meconium ileus. Nonoperative Gastrografin enema or enterotomy-irrigation can relieve obstruction in uncomplicated meconium ileus and avoid an enterostomy in most cases.

Cystic Fibrosis↗
PubMed1989

Increased incidence of delayed gastric emptying in children with gastroesophageal reflux. A prospective evaluation.

Symptomatic gastroesophageal reflux is a common cause of failure to thrive, aspiration, and chronic pulmonary infection in infants. Gastric emptying was prospectively evaluated in 99 infants and children with symptomatic gastroesophageal reflux. Twenty-eight (28.2%) of 99 patients with gastroesophageal reflux had delayed gastric emptying. Twenty-one (75%) of the 28 patients underwent a concomitant gastric drainage procedure at the time of fundoplication. Seven had fundoplication alone and developed symptoms of early satiety, gas bloat, gagging, and pain postoperatively. Medical therapy was ineffective in these patients, and 5 improved after pyloroplasty. Delayed gastric emptying is common in patients with gastroesophageal reflux. These findings suggest that after fundoplication, symptoms of gagging, early satiety, and gas-bloat syndrome may be related to delayed gastric emptying. This implies that a gastric emptying study should be performed preoperatively.

Adolescent↗
PubMed1989

Delayed presentation of a right-sided diaphragmatic hernia and group B streptococcal sepsis. Two case reports and a review of the literature.

Group B streptococcal sepsis was associated with delayed presentation of an unsuspected right-sided diaphragmatic hernia in two neonates. These unusual clinical observations and a review of 24 similar cases from the literature form the basis of this report. Infants present with respiratory distress during the first few hours of life and have group B streptococcal sepsis confirmed by results of blood cultures. The right side of the diaphragm appears normal on the initial chest roentgenogram in the majority of cases. After initial improvement with antibiotic therapy and ventilatory support, sudden deterioration of respiratory status may occur. Subsequent chest roentgenograms often demonstrate herniated viscera in half of the cases, while ultrasound examination, isotopic liver scan, and peritoneography are useful in achieving a diagnosis in the other cases. If recognized, survival is 100% following hernia repair. Persistent respiratory symptoms in a neonate who is recovering from group B streptococcal sepsis should prompt a careful evaluation of the right side of the diaphragm for the presence of an unsuspected posterolateral hernia.

Hernia, Diaphragmatic↗
PubMed1989

The effect of a neurotoxin (benzalkonium chloride) on the lower esophagus.

Achalasia of the esophagus may be associated with abnormalities of the myenteric plexus (hypo- and anganglionosis). This report evaluates this relationship by studying the effect of benzalkonium chloride (BK, a topical neurotoxin) on the lower esophagus. Following midline laparotomy, topical BK (0.5%) was applied to the muscularis of the lower 1.0 cm of the esophagus for 30 min in 38 Sprague-Dawley rats (200 g). Thirty-eight additional rats acted as controls (unoperated, n = 19; sham laparotomy, n = 19). At 1 and 3 months animals were evaluated for weight gain, daily food intake (g/100 gm body wt), lower esophageal sphincter (LES) manometry, and contrast esophagram. At 3 months, the esophagus was evaluated for histologic study and acetylcholinesterase staining. Esophagram showed distal narrowing with proximal dilatation in BK rats (inner diameter 4.71 +/- 0.61 vs 6.17 +/- 0.58 in controls, P less than 0.001). Daily food intake was significantly less in BK rats (5.57 +/- 0.41 g vs controls 7.69 +/- 0.33 g P less than 0.001). Daily weight gain was also less in BK rats (1.13 +/- 0.34 vs controls 1.83 +/- 0.25, P less than 0.001). An increased LES pressure was noted in BK rats (5.45 +/- 0.89 mmHg vs controls 4.04 +/- 1.04 mmHg; P less than 0.1). A histologic study showed aganglionosis in BK rats with positive cholinesterase staining fibers compared to controls. Topical BK results in distal esophageal aganglionosis characterized by distal narrowing, proximal dilatation, decreased food intake, and limited weight gain when compared to controls. These findings are similar to those observed in achalasia and support a primary neurogenic cause for its etiology.

Acetylcholinesterase↗
PubMed1989

Impaired bowel motility following small intestinal transplantation.

Little information is available concerning motility following bowel transplantation and the appropriate timing of offering enteral feedings. Eight Lewis rats (300 g) underwent small bowel transplant as described by R. P. Harmel, Jr., and H. Stanley (J. Pediatr. Surg. 21:214, 1986). Silver oxide electrodes were placed in the native proximal ileum and in the distal segment of the homograft. Four control rats underwent laparotomy and placement of silver oxide electrodes in the jejunum and proximal ileum. Leads were brought out through a stab wound and the abdominal incision was closed. The electrodes were connected to a continuous recorder. Basal electrical rhythm (BER) was recorded periodically and was evaluated daily as the average of three readings per animal per day. Homograft electrical activity was not observed until at least 40 hr post-transplant and never attained the level of BER of the native intestine (P less than 0.05). Myoelectric complex potentials were not observed in the transplanted rats until post-operative day 11. These data suggest that basal electrical activity is significantly impaired following bowel transplantation. This study indicates that intestinal activity can be monitored for extended periods of time and may be a useful method of evaluating recovery of motility post bowel transplant prior to initiating enteral intake.

Animals↗
PubMed1989

Gastroesophageal dysfunction in Cornelia de Lange syndrome.

Children with Cornelia de Lange (CDL) syndrome present with feeding problems related to swallowing incoordination, poor esophageal motility, and gastroesophageal reflux (GER). These abnormalities of esophageal function result in failure to thrive and life-threatening aspiration pneumonia. Severe GER was documented in four patients with CDL syndrome. Three were managed successfully with an antireflux procedure (Nissen fundoplication) and a concomitant feeding gastrostomy. These observations confirm the high incidence of abnormal esophageal function in patients with CDL syndrome, and suggest that an antireflux procedure and feeding gastrostomy are important considerations in their clinical management.

Child, Preschool↗
PubMed1989

Evaluation of high-intensity therapeutic ultrasound irradiation in the treatment of experimental hepatoma.

A study evaluating the efficacy of high-intensity therapeutic ultrasound (HITU) as a treatment modality in experimental hepatoma is reported. Morris hepatoma (3924) 1 x 10(6) cells were transferred subcutaneously into 40 male ACI rats (weight, 150 to 200 g). Animals were divided into four experimental groups: group 1 (n = 10) consisted of untreated controls; group 2 (n = 10) received intraperitoneal cyclophosphamide 50 mg/kg as a single dose; group 3 (n = 10) underwent HITU only; and group 4 (n = 10) received both chemotherapy (as in group 2) and HITU (as in group 3). HITU was administered with a 5.5-cm diameter 4-MHz quartz transducer creating a continuous wave with 400 W/cm2 focal intensity. The entire tumor was irradiated in 1-mm increments (horizontal and vertical) using treatment cycles of 4 seconds on and 11 seconds off. Total body weight and tumor volume were measured on the day of treatment, and 4 weeks later. At 4 weeks, the animals were killed, the tumor was excised and weighed, and tumor volume was determined. Tumor volume in all treated animals (groups 2, 3 and 4) was significantly smaller than in controls (P less than .001) at 4 weeks, and tumor volume for animals in group 4 was significantly smaller than for those in groups 2 and 3 (P less than .01). These data indicate that HITU significantly reduces tumor size when compared with control rats with Morris hepatoma. A synergistic effect of chemotherapy and HITU was observed and resulted in an enhanced tumor response and reduction of tumor size.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1989

Gastrointestinal injuries in childhood: analysis of 53 patients.

Gastrointestinal injuries were noted in 53 children. Blunt trauma was responsible for 51 cases, and penetrating wounds in two. There were 42 boys and 11 girls (mean age, 8.1 years). The site of injury was the stomach (2), duodenum (17), jejunum (19), and ileum (15). Types of injury included two gastric perforations, 16 duodenal hematomas, one duodenal laceration, 27 jejunoileal perforations, five mesenteric avulsions, one abdominal wall laceration and evisceration, and one entrapment necrosis between lumbar vertebrae. Diagnosis was accomplished by observing free air on x-ray, with contrast (duodenal haematoma), computed tomography, and frequent examination (noting peritoneal irritation). Thirty-four associated injuries occurred in 21 patients (40%) including the liver (6), pancreas (6), skeletal injury (6), head trauma (5), diaphragm (4), lung (3), spleen (2), and kidney (2). Nine of 16 duodenal hematomas resolved non-operatively, while seven were evacuated during other procedures. Twenty-three of 30 perforations had simple closure, while seven (jejunoileal) were resected. Mesenteric avulsions required resection in five cases--the eviscerated bowel was replaced and the entrapped bowel resected. Twenty complications occurred in 13 patients, including atelectases (6), pseudocyst (5), sepsis (4), wound infection (2), subhepatic abscess (1), subglottic stenosis (1), and short bowel syndrome (1). One infant (aged 2 months) with a duodenal laceration died of head injuries (1/53 = 1.8% mortality). Prompt recognition and appropriate treatment result in improved survival.

Child↗