Hepatoblastoma and polyposis coli (familial adenomatous polyposis)
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Antineoplastic Combined Chemotherapy Protocols↗
Biomedical subjects
L Spitz
Publications and source records attributed to L Spitz.
Vascular access.
In a retrospective survey of vascular access by means of central venous catheters, those inserted via a tunnel lasted four times longer than those inserted directly into a vein. The latter were four times more likely to become infected. The general health of patients receiving chemotherapy resulted in frequent episodes of sepsis and one-third of all catheters were removed because of presumed infection. There were no complications relating to insertion, which was by direct exposure of a central vein, preferably the right internal jugular. Long-term atrial catheters were not associated with major venous thrombosis or cardiac complications. Safe vascular access is an important contribution to the management of children with malignant disease, notwithstanding the high infection rate. A specially trained nurse, working closely with experienced play leaders and social workers, minimises the technical and psychological problems associated with long-term central venous catheters.
Catheterization, Central Venous↗
Fibrous tissue tumours.
Thirty-three patients, ranging in age from newborn to 12 years, with fibrous tissue tumours, were treated at the Hospital for Sick Children, Great Ormond Street, London, from 1970 to 1984. Seven infants presented with tumours at birth. In 42% of cases, the tumour was noted during the 1st year of life. The anatomical distribution of the lesions was: upper extremity 6, lower extremity 5, head and neck 10, trunk 6, thoracic cavity 2 (heart 1), abdominal cavity 4 (pelvis, bladder, small bowel mesentery, and canal). Three patients had multiple tumours. Seven of the tumours were classified as sarcomas. Complete excision was possible in 28 patients, following initial biopsy in six patients of whom three were treated preoperatively with radiotherapy and/or chemotherapy. There were two deaths, one in an infant with a huge resectable pelvic tumour and the other in a child with cardiac fibroma. Recurrences occurred in 14 patients (48%), in nine of whom the recurrences were multiple. The mean time interval to recurrence was 13.8 months.
Adolescent↗
The importance of oral sodium replacement in ileostomy patients.
The main function of the colon is fluid and sodium conservation. In ileostomy patients these colonic functions are lacking. The consequence is excessive loss of fluid and sodium, failure to thrive, and skin excoriation around the ileostomy. Patients with ileostomies require 6-10 mmol/kg sodium per day. With ordinary feeds, infants receive 2-4 mmol/kg sodium; therefore the sodium deficit may be estimated at 4-6 mmol/kg per day. Monitoring of adequate sodium substitution is best carried out by measuring the concentration of sodium in spot urine. Levels higher than 10 mmol/l sodium signify an adequate oral sodium intake. During the initial period of oral feeding, glucose excretion in the ileostomy fluid must be monitored, as glucose-positive ileostomy effluence necessitates additional sodium substitution in order to activate the sodium and glucose cotransport. Thirty neonates with ileostomies were followed-up retrospectively. All patients received a sodium substitution of at least 4-6 mmol/kg orally per day. The 30 patients had a total of 4769 ileostomy-days. All patients were successfully fed orally and most of them nursed at home until closure of the ileostomy.
Administration, Oral↗
The prevention of post-operative adhesions using a gastrointestinal prokinetic agent.
Attempts have previously been made to reduce adhesion formation by promoting early intestinal motility. The prokinetic agent Cisapride was used in an animal model of end-to-end large bowel anastomoses. Twenty Wistar rats receiving Cisapride 1 mg/kg subcutaneously (sc) twice daily for two days post-operatively were compared with 20 controls receiving saline 0.2 mL in a similar regimen. The points of adhesion of the anastomosis to the following structures were enumerated: tubal fat; mesentery; omentum; caecum; small bowel; and abdominal wall. The treatment group had significantly fewer adhesions: 2.8 +/- 0.9 v 4.3 +/- 1.0. This was mainly in the number of small bowel attachments, 3.8% compared with 37.8%. The study was repeated using small bowel anastomoses. Cisapride again reduced the number of adhesions compared with controls: 2.1 +/- 0.4 v 4.2 +/- 1.4. Cisapride therapy resulted in greater post-operative stool weights and food consumption: control, 3.87 +/- 1.1 stool, 17.04 +/- 4.3 g food; Cisapride 4.43 +/- 0.9 g stool, 19.8 +/- 4.7 g food. The enhanced motility did not affect the anastomotic strength of the small bowel; bursting pressures at a constant inflation rate of 1.1 mL/min were: control 212.8 +/- 56.0 mmHg; Cisapride, 215.8 +/- 58.9 mmHg (NS).
Administration, Cutaneous↗
Surgery, sepsis, and nonspecific immune function in neonates.
An audit of 143 neonatal surgical operations identified low birth weight infants as being at particular risk of developing post-operative sepsis following major surgical trauma. Activity of the nonspecific immune system was examined pre- and post-operatively in order to elucidate the susceptibility to post-operative sepsis of these infants. A chemiluminescence micro-method was developed specifically for this purpose. Neonatal plasma was shown to have significantly impaired opsonising activity, and activity was further reduced in low birth weight infants. Levels of immunoglobulin and complement factors were also reduced, particularly in the low birth weight infant. Following major surgical trauma polymorph activity was significantly lower in low birth weight infants than in mature neonates.
Anti-Bacterial Agents↗
Neonatal gastrointestinal perforation.
During the 7-year period from 1980 to 1986, 56 neonates with gastrointestinal (GI) perforation were treated in the Hospital for Sick Children, London. The overall mortality rate was 30%. The highest mortality rate (60%) was associated with gastric perforation, which in this series occurred only in premature neonates. Colorectal perforation carried the lowest risk of mortality (17%). The mortality rate from small bowel perforation was 35%. Prematurity and low birth weight were significant factors increasing the mortality rate from neonatal intestinal perforation. The predominant cause of perforation in the small and large intestine was necrotizing enterocolitis and the most common site of perforation was the terminal ileum. Mechanical ventilation was thought to be the cause of the perforation in four of the five neonates with gastric perforation. Primary anastomosis is a definite option in neonates who are stable intraoperatively. The mortality rate and in-patient stay for neonates who had primary anastomosis were less than for those who had stomas.
Enterocolitis, Pseudomembranous↗
Pharmacological manipulation of postoperative intestinal adhesions.
Paralytic ileus and intestinal adhesions are common events following intra-abdominal surgery. The theoretical hypothesis 'that stimulation of the postoperative bowel will reduce intestinal adhesions' was studied in a rat model for intestinal adhesions in which postoperative bowel motility was pharmacologically manipulated. Immediate postoperative stimulation of gastrointestinal motility by the prokinetic agent, Cisapride, resulted in a significant reduction in both the number and extent of adhesions. Inhibition of postoperative intestinal motility with the anticholinergic agent, atropine, resulted in a greater number of more dense adhesions involving an increased length of bowel.
Animals↗
Aneurysm of the ductus arteriosus.
Two neonates with aneurysms of the ductus arteriosus were seen. One suffered a fatal spontaneous rupture of the aneurysm, and the second (in which it was an incidental finding) the aneurysm was monitored by echocardiography, and seemed to resolve spontaneously.
Aneurysm↗
Congenital diaphragmatic defects that present late.
Twenty two patients (age range: 1 month to 11 years) were treated for congenital diaphragmatic defects (excluding hiatus hernia) in the six year period 1983-8. Presenting features were failure to thrive (n = 7), abdominal pain and vomiting (n = 4), chronic respiratory symptoms (n = 3), and inability to wean from ventilatory support (n = 3). The defect was an incidental finding in five patients. Operative repair was performed with no mortality or serious postoperative morbidity. Dramatic improvement occurred in 15 of the 17 symptomatic patients. Awareness of the differential diagnosis should avoid delay in diagnosis or inappropriate treatment. Surgical correction is strongly recommended in all cases.
Child↗
Factors associated with developmental progress of full term neonates who required intensive care.
The development of 43 infants born at full term, who were admitted to neonatal intensive care units shortly after birth, was compared in a prospective longitudinal study with that of a group of 29 healthy newborn babies. Thirty infants required emergency operations during the neonatal period, and 13 were admitted for medical reasons. The effects of being in hospital and being separated from their mothers were also studied. At 1 year the 'sick' babies were performing significantly less well in almost all areas of development. In the group of sick infants, the mothers' mental health explained 25% of the variance in developmental outcome at 6 months. At 1 year the most important predictor was length of stay in hospital, which explained 35% of the variance.
Birth Weight↗
Oesophageal atresia and associated anomalies.
Of 253 infants with oesophageal atresia treated over an eight year period, 122 (48%) had a total of 213 other anomalies. Most commonly affected were the cardiovascular (61 cases, 29%), anorectal (30 cases, 14%), and genitourinary (29 cases, 14%) systems. The VATER (or VACTERL) association was present in 10% of cases, but occurred more often in patients who had oesophageal atresia without an associated tracheo-oesophageal fistula (3/13, 23%). The level of the associated anorectal malformation was not associated with the type of oesophageal atresia. The presence and severity of other anomalies did not influence the basic approach to treatment of the oesophageal atresia--that is, primary repair whenever possible. Despite aggressive treatment, cardiac malformations were the most common cause of death. There were five infants with the CHARGE association, two with Potter's syndrome, and two with 'SCHISIS' syndrome (cleft lip and palate, omphalocoele, and hypogenitalism).
Abnormalities, Multiple↗
Gastro-oesophageal reflux and intestinal malrotation in children.
In a series of 74 children undergoing Nissen fundoplication for persistent gastro-oesophageal reflux unresponsive to intensive medical treatment, there was an unexpectedly high incidence (54 per cent, n = 40) of intestinal malrotation. The series was not homogeneous, 25 of the children having reflux as a complication of a serious congenital abnormality (48 per cent incidence of intestinal malrotation), and 49 presenting with 'idiopathic' reflux (57 per cent incidence of intestinal malrotation). Intestinal malrotation is best detected pre-operatively by careful barium radiology, requiring clinicians to be aware of the association. In our experience, in children over the age of 3 months, both an antireflux operation and Ladd's procedure are often necessary to stop gastro-oesophageal reflux when an intestinal malrotation is present.
Adolescent↗
Pancreatic exocrine and endocrine function after subtotal pancreatectomy for nesidioblastosis.
Pancreatic exocrine and endocrine function was assessed in seven patients 1 to 2 years after 95% pancreatectomy (group A) and three patients 9 to 11 years after 75% pancreatectomy (group B). In all cases surgery was undertaken for the treatment of hyperinsulinism and the histologic diagnosis was nesidioblastosis. The activities of pancreatic enzymes and bicarbonate concentrations were generally normal in group B, but were reduced in approximately half the children in group A. One child in group A had significant exocrine failure and poor weight gain. Blood glucose levels and fasting insulin levels were normal during a standard glucose tolerance test in all of the group B patients. One had a low fasting blood glucose level. In the group A patients three had low fasting glucose levels and one a frankly diabetic glucose tolerance test. C peptide and insulin levels were comparable but inappropriate insulin levels were noted in one patient, suggesting that the control of glucose-stimulated insulin release may remain abnormal. The results suggest that pancreatic function is not seriously impaired in the majority of patients 1 to 2 years after 95% pancreatectomy and that it is comparable to that noted in 75% pancreatectomy patients followed over a longer period of time.
Amylases↗
Motor disorders of the oesophagus in gastro-oesophageal reflux.
Mechanisms of gastro-oesophageal reflux were studied by oesophageal manometry and pH monitoring in 33 children: nine controls, 15 with gastro-oesophageal reflux alone, and nine with reflux oesophagitis. A total of 122 episodes of reflux were analysed in detail: 82 (67%) were synchronous with swallowing and 40 (33%) asynchronous. Infants with trivial symptoms had gastro-oesophageal reflux synchronous with swallowing, whereas those with serious symptoms had slower acid clearance and asynchronous reflux. There were significant differences in lower oesophageal sphincter pressure and amplitude of oesophageal contractions between controls and patients with both gastro-oesophageal reflux and reflux oesophagitis. In reflux oesophagitis there was a decrease in lower oesophageal sphincter pressure and the contractions had a bizarre waveform suggesting a neuropathic process.
Adolescent↗
Relationship of birth weight to the pathogenesis of necrotizing enterocolitis in the neonatal piglet.
The relationship between birth weight and the development of necrotizing enterocolitis (NEC) was studied in the term-delivered neonatal piglet. Hypoxia (pO2 less than 30% for 1 h) coupled with hyperviscosity (hematocrit more than 75%), with and without splenectomy, resulted in a high frequency and severity of NEC. This effect was most pronounced in low birth weight (LBW) animals. A statistically significant inverse relationship was found between birth weight and the number and severity of lesions. Splenectomy increased the incidence and severity of lesions particularly in LBW animals. A predominance of lesions was found in the distal ileum with occasional occurrences elsewhere in the small bowel and in the proximal colon. The neonatal piglet is capable of producing the full spectrum of NEC under acceptable experimental conditions. LBW is a significant predisposing factor in the pathogenesis of NEC.
Animals↗
Long-term survival after advanced neuroblastoma.
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Antineoplastic Combined Chemotherapy Protocols↗
Adhesion obstruction following Nissen fundoplication in children.
The incidence of postoperative adhesion intestinal obstruction among 156 children who had undergone Nissen fundoplication for intractable gastro-oesophageal reflux was determined. There were 18 episodes of obstruction in 16 patients (10.3 per cent). The mean interval between fundoplication and adhesion obstruction was 10 months (range 10 days-4 years). Additional procedures performed at the original laparotomy substantially increased the risk of developing obstruction. Relaparotomy for adhesion obstruction was required by 21 per cent of patients who had a Ladd's procedure and 12 per cent who had appendicectomy. Presenting symptoms were not typical of intestinal obstruction because many of these children were unable to vomit. Only three did vomit but all had radiological appearance suggestive of small bowel obstruction. There were two deaths directly related to adhesion obstruction.
Child, Preschool↗