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Biomedical subjects

L Spitz

Publications and source records attributed to L Spitz.

At least 19 recordsLinked to original sources

Laparoscopic surgery in children is associated with an intraoperative hypermetabolic response.

BACKGROUND: Laparoscopic surgery is thought to be associated with a reduced metabolic response compared to open surgery. Oxygen consumption (VO2) and energy metabolism during laparoscopic surgery have not been characterized in children. METHODS: We measured respiratory gas exchange intraoperatively in children undergoing 19 open and 20 laparoscopic procedures. Premature infants and patients with metabolic, renal, and cardiac abnormalities were excluded. Anesthesia was standardized. Unheated carbon dioxide was used for insufflation. VO2 was measured by indirect calorimetry. Core temperature was measured using an esophageal temperature probe. RESULTS: We found a steady increase in VO2 during laparoscopy. The increase in VO2 was more marked in younger children and was associated with a significant rise in core temperature. Open surgery was not associated with significant changes in core temperature or VO2. CONCLUSIONS: Laparoscopy in children is associated with an intraoperative hypermetabolic response characterized by increased oxygen consumption and core temperature. These changes are more marked in younger children.

Adolescent↗

Medical, surgical, and health outcomes of gastrostomy feeding.

A prospective controlled study with repeated measures before and after surgery examined the medical, surgical, and health outcomes of gastrostomy for children with disabilities at a tertiary paediatric referral centre in the North Thames area, UK. Anthropometric measures included weight, mid-upper-arm and head circumference. Five-day prospective food diaries were completed and data on physical health and surgical outcomes recorded. Seventy-six children participated and underwent gastrostomy (44 males, 32 females; median age 3 y 4 mo, range 4 mo-17 y 5 mo), and 35/76 required an anti-reflux procedure. Categories of disability were: cerebral palsy (32/76), syndrome of chromosomal or other genetic origin (25/76), slowly progressive degenerative disease (11/76), and unconfirmed diagnosis (8/76). Most children had gross motor difficulties (99%) and were non-ambulant (83%). Oromotor problems were identified in 78% of children, 69% aspirated, and 65% were fed nasogastrically before surgery. The mean weight before surgery was -2.84 standard deviation score (SDS; SD 2.21, range -9.8 to 3.4). Two-thirds of children achieved catch-up growth postoperatively: weight-for-age (mean difference 0.51 SDS, 95% CI 0.23-0.79, p=0.001) and mid-upper arm circumference (mean difference 1.12 cm, 95% confidence interval 0.50-1.75, p=0.001). Health gains included a reduction in drooling, secretions, vomiting, and constipation. Major surgical complications were found in 13/74 children. The study provides evidence that catch-up growth and health gains are possible following gastrostomy.

Adolescent↗

Ex vivo liver surgery for extraadrenal pheochromocytoma.

A case of a 15-year-old girl with a malignant extraadrenal pheochromocytoma situated between the inferior vena cava and the portal triad is described. Using ex vivo technique, the tumour was successfully resected on the bench, as a previous attempt to remove the tumour in situ had been abandoned. The surgical aspects and implications of ex vivo surgery are discussed, highlighting the increased operative risk, perioperative mortality, and poor long-term results in patients with malignant tumours.

Adolescent↗

Echocardiographic assessment of conjoined twins.

OBJECTIVE: To determine the accuracy of prenatal and postnatal echocardiography in delineating the degree of cardiac fusion, intracardiac anatomy (ICA), and ventricular function of 23 sets of conjoined twins with thoracic level fusion presenting to a single centre over a 20 year period. METHODS: 13 thoracopagus, 5 thoraco-omphalopagus, and 5 parapagus pairs presenting to the authors' institution between 1985 and 2004 inclusive were assessed. Echocardiographic data were analysed together with operative intervention and outcome. Twins were classified according to the degree of cardiac fusion: separate hearts and pericardium (group A, n = 5), separate hearts and common pericardium (group B, n = 7), fused atria and separate ventricles (group C, n = 2), and fused atria and ventricles (group D, n = 9). RESULTS: The degree of cardiac fusion was correctly diagnosed in all but one set. ICA was correctly diagnosed in all cases, although the antenatal diagnosis was revised postnatally in three cases. Abnormal ICA was found in one twin only in two group A pairs, one group B pair, and both group C pairs. All group D twins had abnormal anatomy. Ventricular function was good in all twins scanned prenatally, and postnatally function correlated well with clinical condition. Thirteen sets of twins in groups A-C were surgically separated; 16 of 26 survived. None from groups C or D survived. CONCLUSIONS: Prenatal and postnatal echocardiography accurately delineates cardiac fusion, ICA, and ventricular function in the majority of twins with thoracic level fusion. It is integral in assessing feasibility of separation. The outcome in twins with fused hearts remains dismal.

Echocardiography↗

Resection and primary anastomosis is a valid surgical option for infants with necrotizing enterocolitis who weigh less than 1000 g.

HYPOTHESIS: Primary anastomosis following intestinal resection is a valid surgical option in the treatment of infants with necrotizing enterocolitis (NEC) who weigh less than 1000 g. DESIGN: Retrospective case series. SETTING: Tertiary neonatal surgery referral center. PATIENTS: All infants with confirmed NEC weighing less than 1000 g admitted to our intensive care unit over 4 years. INTERVENTION: For infants requiring laparotomy and intestinal resection, primary anastomosis was performed whenever the clinical condition permitted. MAIN OUTCOME MEASURES: Short- and long-term survival, length of intensive care unit stay, and complications. RESULTS: Fifty-one infants with NEC who weighed less than 1000 g were admitted during the study period. Twelve infants underwent intestinal resection and primary anastomosis (median weight at surgery, 0.83 kg; range, 0.6-0.96 kg). One infant developed recurrent NEC, requiring further surgery, but there were no anastomotic leakages and no strictures. The median postoperative stay on our intensive care unit was 14 days (range, 2-49 days). All 12 infants survived their episode of acute NEC, and 8 are alive, with a median follow-up of 34.2 months (range, 4.7-48.4 months). Only 1 death was related to NEC. During the same period, 14 infants underwent intestinal resection and stoma formation. Ten survived the acute episode, and 6 are alive at a median follow-up of 24.0 months (range, 13.1-33.9 months). The median postoperative intensive care unit stay was 18 days (range, 2-74 days). Necrotizing enterocolitis-related complications occurred in 8 of these infants. CONCLUSIONS: The outcome of infants with NEC who weigh less than 1000 g and undergo primary anastomosis is comparable to that of infants treated using alternative techniques. It is, therefore, a valid surgical option in selected infants. The mortality in this group of infants is high.

Anastomosis, Surgical↗

Peritoneovenous shunting is an effective treatment for intractable ascites.

AIM AND METHODS: A retrospective review was carried out of children undergoing peritoneovenous shunting for intractable ascites. RESULTS: 11 children, aged 3 months to 12 years (median 31 months) underwent peritoneovenous shunting over the past 17 years. The duration of ascites ranged from one month to 2.5 years (median two months). The primary pathology consisted of previous surgery in eight (three neuroblastoma, one renal carcinoma, one hepatoblastoma, one adrenal teratoma, one renal artery stenosis, and one diaphragmatic hernia), and cytomegalovirus hepatitis, lymphatic hypoplasia, and lymphohistiocytosis in one patient each. All patients had failed to respond to previous treatment including peritoneal drainage in six patients, diuretics in five, and parenteral nutrition in five. There were no intraoperative problems. Postoperative complications included pulmonary oedema in three patients, shunt occlusion in three, infection in two, and wound leakage in one. Ascites resolved after shunting in 10 patients. Five shunts were removed one to three years after insertion without recurrence of ascites. Three others are free of ascites with shunts in place for less than one year postoperatively. Three children died from their underlying disease: two after resolution of ascites (neuroblastoma) and one in whom the ascites failed to resolve (lymphohisticytosis). CONCLUSIONS: Peritoneovenous shunting is an effective treatment for symptomatic intractable ascites in children (10 of 11 successful cases in this series). Elective removal of the shunt after one year is recommended.

Ascites↗

H-type tracheo-oesophageal fistula with congenital oesophageal stenosis.

H-type tracheo-oesophageal fistula (H-TOF) and congenital oesophageal stenosis (COS) are both rare anomalies. Only six cases of H-TOF in association with COS have been described in the literature. Characteristically, diagnosis is delayed, often well beyond the neonatal period. We report a patient who was accurately diagnosed on day 12 of life, and outline several of the key clinical dilemmas encountered in managing a neonate with this combination of rare congenital anomalies.

Esophageal Stenosis↗

Seamless management of biliary atresia in England and Wales (1999-2002).

BACKGROUND: Before 1999, infants born in the UK with suspected biliary atresia were investigated in regional centres, and, if confirmed, a Kasai operation was done there. Since 1999, all infants with suspected biliary atresia in England and Wales, UK, have been referred to one of three designated centres where both the Kasai operation and liver transplantation (if necessary) could be done. METHODS: We assessed clearance of jaundice (bilirubin <20 micromol/L) as an early outcome in all cases of biliary atresia referred from one of the three centres. We then estimated survival using the Kaplan-Meier method with endpoints of liver transplantation or death. FINDINGS: 148 infants with biliary atresia were treated between January, 1999, and June, 2002. A primary portoenterostomy was done in 142 (96%) infants and a primary liver transplant in five (3%). One child died before any intervention. Early clearance of jaundice after portoenterostomy was achieved in 81 of 142 (57%) infants. Liver transplantation was done in 52 (37%) of those undergoing portoenterostomy. 13 (9%) infants died. Of the 135 children who survived, 84 (62%) still have their native liver and 51 (38%) had transplantation. The median follow-up of survivors was 2.13 (range 0.5-4.1) years. The overall 4-year estimated actuarial survival was 89% (95% CI 82-94). The 4-year estimated actuarial survival with native liver was 51% (42-59%). INTERPRETATION: Our early results suggest that surgical outcome can be improved by centralisation of care to supra-regional centres.

Biliary Atresia↗

Outcome and complications after resection of hepatoblastoma.

PURPOSE: The aim of this study was to review the outcome and complications after resection of hepatoblastoma treated over 2 decades in our institution. METHODS: Clinical, radiologic, and pathologic data were reviewed retrospectively, focusing on the outcome and complications. RESULTS: Between January 1978 and December 2002, 56 children were treated for hepatoblastoma. The age range was 0.08 to 8.74 years (median, 1 year). The right lobe was involved in 48%, the left lobe in 22%, and in 29% the main bulk of the tumour was centrally located. Surgical procedures included the following: hemihepatectomy in 62%, trisegmentectomy in 18%, extended hemihepatectomy in 16%, and liver transplantation and laparotomy in one patient each. Intraoperative complications occurred in 5(9%)--rupture of the tumour (1), haemorrhage from the contralateral lobe (1), a defect in the left hepatic duct (1), cardiac arrest from tumour embolus (1), and bleeding from the inferior vena cava (1). The mean blood loss was 280 mL (50 to 2,000 mL). Postoperative complications occurred in 12 patients (22%) including subphrenic abscess (3), adhesion obstruction (2), ischaemic stenosis of the bile duct (1), abdominal wound dehiscence (1), pyloric obstruction (1), and pleural effusion (2). Fifteen patients died, 14 as a result of tumour recurrence (mortality rate, 27%).

Antineoplastic Combined Chemotherapy Protocols↗

A histological study of the hph-1 mouse mutant: an animal model of phenylketonuria and infantile hypertrophic pyloric stenosis.

AIM: To quantify the chronological sequence of changes in the morphology and immunoreactivity for neurotransmitters in the pylorus of an animal model of infantile hypertrophic pyloric stenosis and phenylketonuria. METHOD: Thirty specimens of pylorus from hph-1 mice and age/sex matched controls (age range: 10-180 days) were examined using conventional histology and immunohistochemistry for a variety of antigens: protein gene product 9.5, a pan neuronal marker; vasoactive intestinal polypeptide; nitric oxide synthase two antigens coalesced to the same inhibitory neurons in humans; substance P, a potent excitatory neurotransmitter; and calcitonin gene related peptide, a neurotransmitter implicated in the somatic afferent innervation of the stomach. The changes in the morphology of the muscle layers were quantified and statistically analysed for each age group (10, 20, 40, 90 and 180 days). RESULTS: Between 10 and 90 days of age, all muscle layers of the hph-1 mice were hypertrophied, for example, 10 days, hph-1 longitudinal muscle mean diameter = 3.4, control = 1.8; hph-1 circular muscle width = 11.5, control = 4.7. The hph-1 mice were significantly smaller during this period (40 days, hph-1 weight = 10 g, control = 25 g). There was no change in the pattern of expression of the antigens examined within the hph-1 mice compared with the controls. CONCLUSION: Hph-1 mice develop a transient smooth muscle hypertrophy of the pylorus attended by gastric distension and failure to gain weight. These changes resolve as the pyloric muscle hypertrophy resolves.

Age Factors↗

Myocardial carnitine palmitoyltransferase I as a target for oxidative modification in inflammation and sepsis.

CPT I (outer membrane carnitine palmitoyltransferase I) is a crucial enzyme in myocardial substrate selection. Two isoforms exist in the heart, the liver (L-) and muscle (M-) isoforms, which have different kinetic characteristics and alter in relative amounts during the neonatal/weaning/adult transition. CPT I is a point for control and regulation of fatty acid oxidation via modulation of its activity by malonyl-CoA, the concentration of which is set by acetyl-CoA carboxylase, AMP-activated protein kinase and malonyl-CoA decarboxylase in response to, for example, alterations in glucose supply. Systemic inflammatory responses and sepsis lead to myocardial dysfunction as part of multiple system organ failure. We have shown that: (i) myocardial CPT I activity is inhibited during neonatal sepsis; (ii) on the basis of inhibitor studies this inhibition appears to be of M-CPT I rather than L-CPT I; (iii) nitration of M-CPT I occurs, probably by peroxynitrite, and this may be responsible for the decrease in CPT I activity; (iv) myocardial CPT I activity is also inhibited in another model of systemic inflammatory response, namely intestinal ischaemia/reperfusion injury, but this can prevented by whole-body moderate hypothermia. Inhibition of M-CPT I would be predicted to alter myocardial substrate selection but there are several questions that remain to be answered.

Animals↗

The histological appearances of Nissen-type fundoplication in the ferret.

Nissen fundoplication is of proven effectiveness in the surgical control of gastro-oesophageal reflux. However, our understanding of the effects of fundoplication upon foregut physiology is incomplete and post-operative symptoms are often poorly understood. This experimental study aimed systematically to characterize the tissue response to fundoplication in an animal model, to improve understanding of the effects of anti-reflux surgery upon foregut physiology. Nissen-type fundoplication was performed in the ferret, and the tissue response at 3 months examined histologically. Sham-operated animals that underwent laparotomy but no dissection or wrap, acted as controls. In fundoplicated animals, serosal fibrosis was observed in the gut wall, with patchy replacement of muscle by fibrous tissue. The ventral and dorsal vagal nerve trunks were identified intact within the wrap. In cases where the wrap had spontaneously disrupted, fibrosis was more extensive and there was evidence of nerve damage. This is the first systematic description of the histopathological response to Nissen fundoplication. In the intact wrap, the vagal trunks appear spared, but there is fibrosis in the serosa, extending into the muscularis of the distal oesophagus and region of the cardia. These findings are discussed in relation to the effects of Nissen fundoplication upon gastric physiology and postoperative symptoms.

Animals↗

Gastroesophageal reflux.

Gastroesophageal reflux is common in infants and generally resolves spontaneously within the first year of life as the lower esophageal sphincter mechanism matures. The reflux is only considered a "disease" (GERD) when it becomes symptomatic or causes pathological consequences. GERD is commonly associated with esophageal atresia and there is a high incidence in neurologically impaired children; in both groups conservative treatment is notoriously ineffective. The diagnosis of GER is made on upper gastrointestinal contrast studies, endoscopy and pH monitoring. Medical management comprises antacids, reduction of gastric acid production and prokinetic agents. The indications for antireflux surgery include an established esophageal stricture, associated anatomical defect and failure of medical therapy. Apnoeic episodes secondary to documented GER in the infant, constitute an absolute indication for early surgery.

Child↗

Surgical complications of pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy.

PURPOSE: The aim of this study was to review the surgical complications of pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI). METHODS: A retrospective review was conducted of patients undergoing pancreatectomy for PHHI in one institution over the past 13 years. RESULTS: The records of 48 patients were reviewed; the age at operation ranged from 10 days to 30 months (median, 8 weeks). Weight at operation ranged from 1.97 to 11.4 kg (median, 5.2 kg). There were no deaths. Intraoperative complications comprised bleeding in 7, (major in 2), splenic injury in one, bile duct injury in 2 (1 oversewn, 1 choledochoduodenostomy), and 1 small bowel injury. Postoperatively, 5 children underwent choledochoduodenostomy: 2 for biliary leak and 3 for delayed bile duct stricture. Other postoperative complications included wound infection (n = 3), prolonged ileus (n = 1) and adhesion obstruction (n = 1), and wound leakage (n = 1). Renal failure developed in one child owing to acute tubular necrosis. Nine patients required further pancreatic resection because of continued hypoglycaemia. Three patients continued to require medication for hyperinsulinism despite surgery, 20 required insulin, and 13 required pancreatic enzyme replacement at the time of the last review. CONCLUSIONS: Pancreatectomy resulted in resolution of hyperinsulinism in 45 of 48 patients. Sixteen patients required no further surgery or medication. Pancreatectomy for PHHI may be associated with major intra and postoperative morbidity.

Bile Ducts↗