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Biomedical subjects

L Spitz

Publications and source records attributed to L Spitz.

At least 55 records · Page 3Linked to original sources

Retching and vomiting in neurologically impaired children after fundoplication: predictive preoperative factors.

BACKGROUND/PURPOSE: In neurologically impaired children, retching and recurrent vomiting are common after Nissen fundoplication. The aim of this study was to identify whether there are preoperative factors that predict their occurrence. METHODS: Twenty neurologically impaired children (8 boys, 12 girls; age range, 3 months to 8 years) were studied prospectively by taking a detailed history of behaviors and symptoms associated with feeding before and after Nissen fundoplication for gastroesophageal reflux. RESULTS: Preoperatively, children could be classified into 2 groups. Children in group A had symptoms suggestive of only gastroesophageal reflux (effortless "vomiting" or regurgitation), whereas children in group B exhibited one or more features associated with activation of the emetic reflex (pallor, sweating, retching, forceful vomiting). Postoperatively 0 of 8 in group A retched compared with 8 of 12 in group B (P <.005, Fishers Exact test). CONCLUSIONS: Children at high risk of retching, and ultimately vomiting, after antireflux surgery may be identified clinically preoperatively. They have symptoms that are specifically caused by activation of the emetic reflex rather than to gastroesophageal reflux. In these cases, antireflux surgery could be considered inappropriate and hence be avoided.

Central Nervous System Diseases↗

Energy metabolism of infants and children with systemic inflammatory response syndrome and sepsis.

OBJECTIVE: To evaluate whether critically ill children with systemic inflammatory response syndrome (SIRS) or sepsis have altered resting energy expenditure (REE) and substrate utilization. SUMMARY BACKGROUND DATA: Studies in adults with sepsis have shown increased energy expenditure and mobilization of endogenous fat. In infants and children, energy metabolism and substrate utilization during sepsis have not been characterized. METHODS: Metabolic studies were performed in 21 critically ill children with SIRS or sepsis. Twenty-one stable control children, matched for weight, were also studied. Seven patients required inotropic support and 17 received mechanical ventilation. Fifteen patients with SIRS had evidence of bacterial, fungal, or viral infection and were considered septic. Respiratory gas exchange was measured by computerized indirect calorimetry for 1 to 2 hours continuously. RESULTS: The REE of patients with SIRS or sepsis was not different from that of controls. Similarly, there were no differences in carbon dioxide production and oxygen consumption. Resting energy metabolism was not different between patients with SIRS and patients with sepsis. In addition, the presence of low platelet count or inotropic support did not affect resting energy metabolism. The median respiratory quotient of patients with SIRS or sepsis was 0.88 (range 0.75-1.12), indicating mixed utilization of fat and carbohydrate; this was not significantly different from that of controls. The Pediatric Risk of Mortality Score was not significantly correlated with REE or respiratory quotient. CONCLUSIONS: The energy requirements of children with SIRS or sepsis are not increased. Their resting metabolism is based on both carbohydrate and fat utilization. The authors speculate that these children divert the energy for growth into recovery processes.

Case-Control Studies↗

Antenatal identification of a cervical oesophageal duplication.

Duplications of the alimentary tract can occur from mouth to anus, and often are undetected until they present with complications. We report duplication of the cervical oesophagus presenting as dyspnoea and stridor in a two year old. In retrospect, this had been detected on ultrasound antenatally, but no specific diagnosis had been made. We review the present literature to raise awareness of duplication as one cause of cysts identified antenatally in this region.

Child, Preschool↗

Nitric oxide inhibits neonatal hepatocyte oxidative metabolism.

BACKGROUND/PURPOSE: Liver function is frequently impaired in neonates with sepsis. Nitric oxide (NO) is thought to be a mediator of organ dysfunction and liver oxidative metabolism during sepsis. The authors developed an in vitro model to investigate the effect of NO and the combined effect of NO plus H2O2 on neonatal hepatocyte oxidative metabolism. METHODS: Hepatocytes were isolated from neonatal rats. Oxygen consumption was measured polarographically. In Study A, cells were exposed to S-Nitroso-N-acetylpenicillamine (SNAP), an NO donor, at various concentrations. In study B, myxothiazol and oligomycin, inhibitors of mitochondrial respiration, were added to investigate the site of action of NO. In study C, hepatocytes were incubated in the presence of both SNAP (300 micromol/L) and H2O2 (1.5 mmol/L). In study D, morphological alterations induced by NO and NO plus H2O2 were investigated by hepatocyte electron microscopy. RESULTS: In study A, SNAP caused a dose-dependent decrease in oxygen consumption. A significant inhibition was reached at 300 micromol/L SNAP. In study B, the lack of further inhibition when SNAP was given together with myxothiazol indicates that NO acts intramitochondrially. Similarly, no further inhibition occurred when the NO donor was given together with oligomycin, suggesting that the effect of NO is mainly at the level of ATP synthase. In study C, concomitant addition of 300 micromol/L SNAP and 1.5 mmol/L H2O2 to hepatocytes caused further inhibition of oxygen consumption compared with either SNAP or H2O2 alone. In study D, mild alterations in hepatocyte morphology were noted in the presence of SNAP or SNAP plus H2O2. CONCLUSIONS: In neonatal hepatocytes, NO significantly inhibits mitochondrial oxygen consumption, possibly at the level of ATP synthase. The effect of NO is additive to that of H2O2. Morphological findings were consistent with these biochemical effects and suggest that NO and H2O2 are important mediators of liver damage during sepsis.

Adenosine Triphosphate↗

The management of oesophageal atresia in neonates with right-sided aortic arch.

BACKGROUND/PURPOSE: The management of oesophageal atresia (OA) in association with a right-sided aortic arch (RAA) is challenging. It is controversial whether right or left thoracotomy is the most appropriate surgical approach. The aim of this study was to determine the prevalence of RAA in patients with OA and the most appropriate surgical approach. METHODS: The case records of all the neonates with OA treated over an 18-year period (1980 through 1997) were reviewed. Patients with RAA were analysed with particular reference to the preoperative investigations, operative approach and findings, and postoperative complications. RESULTS: Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of these infants had major cardiac anomalies. The diagnosis was not suspected on preoperative chest x-ray in any of these infants. In only 1 of the 5 neonates who had a preoperative echocardiogram was RAA suspected. A right thoracic approach was performed in the 11 infants in whom a RAA was not suspected. Immediate conversion to a left thoracotomy with primary anastomosis was carried out in 2 infants. In 2 further infants, fistula ligation alone was performed because of a long gap atresia. Anastomosis via the right thoracotomy was performed in 7 infants. There were 3 anastomotic leaks, 1 intractable stricture, and 1 recurrent fistula. Three patients (25%) had a double aortic arch, and, as a result, a left thoracotomy had to be abandoned in 2. There were 3 deaths, 2 as a result of major cardiac anomalies and 1 related to prematurity and sepsis. CONCLUSIONS: RAA occurs in 2.5% of infants with OA. Preoperative chest x-ray and echocardiogram are unreliable as diagnostic modalities. Anastomosis via a right thoracotomy is associated with a 42% leak rate. The presence of a double aortic arch can make the oesophageal anastomosis via a left thoracotomy as difficult as via a right thoracotomy.

Abnormalities, Multiple↗

Nissen-type fundoplication and its effects on the emetic reflex and gastric motility in the ferret.

Recurrent vomiting with failure to thrive is a common problem in neurologically impaired children. Many undergo fundoplication to control the underlying gastro-oesophageal reflux. The results of surgery are not always satisfactory and post-operative retching may be a major problem - a symptom indicative of activation of the emetic reflex. An animal model of antireflux surgery has been developed and used to investigate the effects of such surgery upon the emetic reflex and vagal influences on gastric motility. Following surgery, animals responded to a previously subemetic dose of a centrally acting opiate receptor agonist (loperamide), suggesting that fundoplication may sensitize the emetic reflex. A gastric vago-vagal reflex (tonic inhibition of corpus tone) and responses to direct stimulation of vagal motor efferents (both cholinergic and nonadrenergic noncholinergic responses) were not significantly affected by antireflux surgery. Mechanisms by which neural damage may sensitize the emetic reflex are discussed, together with the possible clinical implications for the management of post-operative symptoms in neurologically impaired children.

Animals↗

Amino acids counteract the inhibitory effect of fentanyl on hepatocyte oxidative metabolism.

BACKGROUND/PURPOSE: Hypothermia is common after major surgery in newborns and can be triggered by intraoperative fentanyl analgesia. Recent studies have found that fentanyl inhibits hepatocyte mitochondrial oxidative metabolism, which is proportional to thermogenesis. In adults it has been shown that amino acids have a thermogenic effect, although the biochemical basis of this phenomenon is not known. The aim of this study was to test the hypothesis that amino acids counteract the inhibition of neonatal hepatocyte oxygen consumption by fentanyl. METHODS: Hepatocytes were isolated from suckling rats, and O2 consumption was measured polarographically. In experiment A hepatocytes were incubated with (1) palmitate alone (control), (2) palmitate plus fentanyl, (3) palmitate plus fentanyl plus amino acids, and (4) palmitate plus amino acids. In experiment B the effects of essential and nonessential amino acids were tested separately. In experiment C, to investigate whether the effect of amino acids is intramitochondrial, hepatocytes were incubated with amino acids plus inhibitors of mitochondrial respiration. RESULTS: In experiment A, fentanyl significantly inhibited O2 consumption (P = .006). This inhibition was reversed by amino acids (P < .001). In experiment B, both essential and nonessential amino acids reversed the effect of fentanyl (P < .001). In experiment C, there was no difference in O2 consumption in the presence of myxothiazol among the groups indicating that amino acids affect intramitochondrial O2 consumption. CONCLUSIONS: (1) Amino acids abolish the inhibitory effect of fentanyl on hepatocyte oxidative metabolism. (2) Amino acids affect intramitochondrial O2 consumption and therefore thermogenesis. (3) Perioperative administration of amino acids in neonates may help to prevent hypothermia and its deleterious effects.

Adjuvants, Anesthesia↗

Intestinal metabolism after ischemia-reperfusion.

PURPOSE: This study explores the effects of ischemia-reperfusion on various metabolic aspects of the small intestine. METHODS: Intestinal ischemia-reperfusion was obtained by clamping and unclamping the superior mesenteric artery in adult rats. Four groups of animals were studied: (A) sham operation for 150 minutes, (B) 90-minute intestinal ischemia, (C) 150-minute intestinal ischemia, and (D) 90-minute intestinal ischemia followed by 60-minute reperfusion. Body temperature was maintained at normothermia (36.5 to 37.5 degrees C). Concentrations of intestinal glucose, succinate, lactate, amino acids, phosphocholine (PC), glycerophosphocholine (GPC), choline, and phosphoenergetics were measured using magnetic resonance spectroscopy of freeze-clamped small intestine extracts. RESULTS: Intestinal ischemia (groups B and C) alone caused a significant drop in glucose and phosphoenergetics but caused an increase in amino acids, succinate, and lactate. Ischemia and ischemia-reperfusion decreased PC and GPC but increased choline. After intestinal reperfusion (group D), no recovery of phosphoenergetics was observed, but there was partial recovery of glucose, succinate, lactate, and amino acids. CONCLUSIONS: There is no recovery of phosphoenergetics after 90 minutes of intestinal ischemia followed by 60 minutes of reperfusion. Partial recovery of glucose, succinate, lactate, and amino acids may reflect equilibration of these metabolites between damaged cells and extracellular fluid.

Amino Acids↗

Pelvic neuroblastoma: low mortality and high morbidity.

BACKGROUND/PURPOSE: Cervical, thoracic, and pelvic neuroblastomas are regarded as having a better outcome than abdominal primaries. The aim of the study was to analyze the results of treatment of pelvic neuroblastomas in our institution. METHODS: The authors reviewed the records of 284 patients with neuroblastoma treated in our hospital during the period 1983 through 1998 and identified 17 (6%) with pelvic tumors. The revised International Neuroblastoma Staging System was used. RESULTS: There were 6 patients with stage 1 disease, 8 with stage 2, 2 with stage 3, and 1 with stage 4 disease. Intraspinal extension of the tumor was present in 7 patients (41%). Except for one child with stage 4 disease, all patients underwent an attempt of tumor excision, and 6 had a complete resection. All 7 patients with intraspinal tumor survived. Permanent postoperative neurological complications occurred in 6 patients (35%). These included sciatic nerve palsy, urinary and fecal incontinence, neuropathic bladder, and leg weakness or nerve root injury L4-S1. Three of 17 patients died, but 1 fatality was unrelated to the tumor. The overall survival rate was 82% and was not influenced by the completeness of tumor resection. CONCLUSIONS: The survival of nonmetastatic pelvic neuroblastoma in our institution is good despite incomplete tumor resection. Intraspinal extension is not a negative prognostic factor. Considering the high incidence of permanent neurological damage after surgery and the generally favorable biological characteristics of these tumors, surgical treatment should not be overaggressive.

Antineoplastic Combined Chemotherapy Protocols↗

Cysts and tumors of the pancreas.

Both pancreatic cysts and pancreatic tumors rarely occur in childhood. However, when encountered they can present a diagnostic and therapeutic challenge to the pediatric surgeon. The aim of this review is to discuss the different types of pancreatic cysts and tumours that may be encountered in the pediatric population, to note the modes available for their diagnosis, and to outline treatment options.

Adenocarcinoma↗

Early complications of gastric transposition operation.

Gastric transposition was performed in 100 children as a definitive procedure for oesophageal replacement between 1982 and 1997 for 69 oesophageal atresia (41 with distal tracheooesophageal fistula, 20 isolated oesophageal atresia and 8 with proximal tracheooesophageal fistula), 16 severe caustic stricture, 7 intractable peptic reflux stricture and 8 miscellaneous causes. Six mortalities were recorded. Sixty-five patients had complications postoperatively and respiratory complication was the most common complication especially in oesophageal atresia patients. Swallowing difficulty, particularly in oesophageal atresia, occurred in 21 per cent of the patients. Ten patients developed cervical leakage with spontaneous closure and 8 patients suffered from anastomosis stricture. Six jejunostomy revisions were required. Three of five pyloromyotomy obtained inadequate gastric drainage post gastric transposition and required the conversion to pyloroplasty. Because of the distinctive low major life-threatening morbidity and low mortality, we concluded that gastric transposition was a safe, easy and preferable procedure for oesophageal replacement in children.

Adolescent↗

Oesophageal atresia, related malformations, and medical problems: a family study.

Oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF) are life-threatening malformations of generally undefined cause. Previous reports of familial cases suggest a genetic contribution. The pattern of inheritance appears non-Mendelian, i.e., multifactorial. Individuals with OA/TOF often have other malformations and medical problems. The aim of this study was to determine the association in OA/TOF cases and healthy control subjects of associated malformations, midline defects, and medical conditions. We also investigate the relationships of these conditions in the relatives of the cases and controls. The results show that infants with OA/TOF frequently have VACTERL anomalies (vertebral, 17%; anal, 12%; cardiac, 20%; renal, 16%; limb, 10%) and other midline defects (cleft lip and palate, 2%; sacral dysgenesis, 2%; urogenital anomalies, 5%). The following medical problems were also reported: oesophageal dysmotility, 21%; gastro-oesophageal reflux, 22%; chest infections, 6%; and autonomic dysfunction, 0.5%. The first-degree relatives of children with OA are much more likely to have one of the aforementioned malformations or medical conditions when compared with the control group: one or more VACTERL anomalies (P < 0.01), gastro-oesophageal reflux (P < 0.05), recurrent respiratory infections (P < 0.05), and autonomic dysfunction (P < 0.001). The more distant relatives also show an increased incidence of such problems although in this case the data must be viewed with caution. The results confirm that the associated malformations and related medical problems occur significantly more frequently in the relatives of individuals with OA/TOF. These families may prove valuable for linkage analysis in an attempt to determine the genetics of OA/TOF.

Abnormalities, Multiple↗

Abnormal enteric nerve morphology in atretic esophagus of fetal rats with adriamycin-induced esophageal atresia.

Gastroesophageal reflux is common in children after successful repair of esophageal atresia (EA), and may be related to a congenital neuronal abnormality of the esophagus. This study employed a fetal rat model of adriamycin-induced EA to investigate whether the innervation of the esophagus is abnormal in EA. The fetal rats were divided into four groups: (1) normal controls; (2) a saline-injected controls; (3) adriamycin administered but without the development of EA; and (4) adriamycin-induced EA. The distal esophageal segments were immunostained with a general neural marker, protein gene product 9.5 (PGP). Immunoreactivity per cross-sectional area (/xsa) was measured with an image analyzer. The extent of the esophageal circumference encircled by PGP-stained nerve tissue was assessed. While there was no significant difference in PGP immunoreactivity/xsa between the groups, the near-complete ring of nerve tissue along the plane of the myenteric plexus was replaced by clusters of nerve tissue in the atretic group (normal vs EA, P = 0. 001, Mann-Whitney U test). The abnormal distribution of nerve tissue in the atretic esophagus may be contributing factor in the esophageal dysmotility seen in EA.

Abnormalities, Drug-Induced↗

Sacral dysgenesis associated with terminal deletion of chromosome 7q: a report of two families.

UNLABELLED: Most cases of sacral dysgenesis are considered to be sporadic events. We present two families in whom the presence of associated clinical features prompted specific investigation of chromosome 7, leading to the identification of an underlying chromosome 7q deletion causing sacral dysgenesis. All affected individuals had microcephaly and developmental delay. Detailed cytogenetic studies confirmed that all three affected individuals had a deletion of chromosome 7q associated with their sacral dysgenesis, developmental delay and related problems. The three affected patients were studied clinically, radiologically and cytogenetically. Eleven unaffected individuals from the two families were also investigated by genetic studies, specifically evaluating chromosome 7. CONCLUSION: It is important that detailed family history, evaluation of associated malformations and the overall clinical picture be considered in identifying the underlying diagnosis in cases of anal stenosis/sacral agenesis. The cases we present demonstrate the value of detailed chromosome studies in such situations.

Abnormalities, Multiple↗