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Biomedical subjects

L Spitz

Publications and source records attributed to L Spitz.

At least 109 records · Page 6Linked to original sources
PubMed1997

Staged pull-through for rectosigmoid Hirschsprung's disease is not safer than primary pull-through.

Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through (staged PT). During the past decade immediate definitive operation (primary PT) has been reported by several investigators with a complication rate ranging from 0% to 20%. In the authors' institution primary PT has been performed since 1992 in patients with rectosigmoid aganglionosis responsive to rectal irrigations. The authors reviewed the records of all patients (n = 124) with Hirschsprung's disease diagnosed and treated between 1989 and 1995. One hundred sixteen patients underwent a definitive operation. This study analysed the complications observed in 87 of these patients with aganglionosis limited to the rectosigmoid colon. Patients were divided into groups according to the type of surgical treatment (staged versus primary), the year of operation (1989 through 1992 versus 1992 through 1995), and the age at primary PT (15 infants age < or = 4 months versus 10 children age > 4 months). There were no deaths. There was no significant difference in complication rates between staged PT and primary PT. Similar complication rates were encountered in infants and children who underwent primary PT. The rate of postoperative complications did not change according to the year of the operation. Major complications were observed in all 3 patients who had a primary Swenson PT compared with 20% who had a primary Duhamel PT. Staged Swenson and Duhamel PT had similar complication rates. The authors conclude that (1) staged PT for rectosigmoid Hirschsprung's disease is not any safer than primary PT; (2) primary PT can be performed safely in young infants; and (3) primary Swenson PT is less satisfactory than primary Duhamel PT.

Child↗
PubMed1997

Markedly improved survival in malignant sacro-coccygeal teratomas--16 years, experience.

AIM OF STUDY: To see the impact of cisplatin- and carboplatin-based protocols on survival in malignant sacrococcygeal teratomas (SCT). METHODS: Twenty infants and children with malignant SCT were treated at the Hospital for Sick Children, Great Ormond Street, London, over a 16-year period from 1979 to 1994. There were 5 males and 15 females, age ranged from 1 day to 3.5 years (mean 18 months). RESULTS: Twelve patients (60%) had distant metastasis: 9 lung metastasis, 2 liver involvement, 2 bony metastases, 1 cerebral metastasis and 1 bilateral inguinal lymph node deposits. From 1982 to 1986, patients received the BEP chemotherapy protocol which included cis-platinum, bleomycin and etoposide (VP 16). After 1986, cis-platinum was replaced with carboplatin in the new JEB protocol. Patient 1 did not receive any chemotherapy, Patients 2-4 received varying protocols (2 deaths), Patients 5-8 received the BEP regime (1 death) and Patients 9-20 received JEB (1 death). The first three deaths were due to uncontrolled local disease and/or metastasis, while the latter death was due to bleomycin toxicity. Overall, 9 of 12 (75%) patients with distant metastasis survived as opposed to 7 of 8 (88%) patients with localised disease. Of the 12 patients who received the JEB protocol, 11 (92%) survived, including 7 patients with metastatic disease and 2 with local recurrence. Seven patients (35%) had relapse while on treatment or follow-up, 4 of these are disease-free with further therapy. In 6 of these children, serum AFP rose before there was clinical or radiological evidence of relapse. In 2 other patients, further chemotherapy was recommenced solely on the basis of rising serum AFP, these patients did not subsequently develop overt metastasis. CONCLUSIONS: We conclude that the treatment of choice for malignant SCT is the JEB regime, to be given for 4 courses or to be continued for 2 courses beyond documented Complete Response (CR). Excision of the primary tumour and coccyx should be done in all cases even if a CR has been documented. Metastases not responding to chemotherapy would need appropriate surgery, radiotherapy is hardly ever needed. An overall cure rate exceeding 90% can be expected.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1997

Meconium ileus: a ten-year review of thirty-six patients.

Of 36 neonates with meconium ileus secondary to cystic fibrosis treated over a 10-year period, twenty-one (58%) had simple uncomplicated disease while fifteen (42%) had complications which included perforation (5), volvulus (6) and atresia (5). Gastrografin enema was employed in 20 infants with relief of obstruction in 8 (40%). Operative procedures consisted of resection and primary anastomosis in seventeen patients, stomas were fashioned in six, three had an enterotomy with irrigation only and two had Bishop-Koop enterostomy. Post-operative complications developed in 5 (18%) of these 28 patients. The overall survival rate was 97%. The one death occurred in an infant with short bowel syndrome, patent ductus arteriosus, hydrocephalus and pulmonary damage. There were eight additional patients who had meconium obstruction in the absence of cystic fibrosis.

Cystic Fibrosis↗
PubMed1997

Acute abdomen. The history.

Correct decision, when managing the acute abdomen in children, are based on careful clinical assessment. An accurate history and a detailed examination will provide more information than a multitude of investigations. The chronological order to appearance and the progression of each individual symptom must be obtained. Consideration of these dynamic changes will often yield an accurate diagnosis. This article outlines a structured approach to history taking and details the common causes of acute abdomen in childhood.

Abdomen, Acute↗
PubMed1996

Intestinal glycosaminoglycans in neonatal necrotizing enterocolitis.

Advanced necrotizing enterocolitis (NEC) is a common neonatal surgical emergency of unknown aetiology. Despite improvements in the prognosis, the aggressive form of the disease is still associated with significant rates of morbidity and mortality. Recent evidence indicates that the extracellular matrix (ECM) is important in gastrointestinal development and glycosaminoglycans, major constituents of the ECM, are attenuated in inflammatory bowel disease. The hypothesis of this study was that changes in the nature and distribution of intestinal glycosaminoglycans occur in NEC. The distribution and nature of glycosaminoglycans were determined in 31 sections of well preserved resection margins and severely diseased bowel from eight neonates affected by NEC. An established histological method of glycosaminoglycans analysis using cationic gold with silver enhancement was employed in this study. The identity of specific glycosaminoglycans was also elucidated using a combination of cationic gold staining and glycanase digestion. In well preserved tissue, staining was seen throughout the full thickness of the bowel. The epithelial basement membrane and basolateral surfaces, lamina propria and submucosa were particularly prominent. In moderate disease, patchy loss of anionic sites was frequently observed with glycosaminoglycans-deficient areas adjacent to intact sites. In severe NEC, there was extensive loss of glycosaminoglycans in most of the sections examined. Glycanase analysis revealed that the glycosaminoglycans in well preserved tissue were sensitive to chondroitinase ABC and only vascular sites were sensitive to heparinase III. The consequences of glycosaminoglycans loss in NEC as demonstrated in this study are not known but modulation of gastrointestinal glycosaminoglycans could be important in the pathogenesis of NEC and may underlie some of the clinical manifestations of this condition.

Colon↗
PubMed1996

Conjoined twins.

Explore the source record for details and available documents.

Female↗
PubMed1996

Idiopathic constipation is not associated with increased NCAM expression on intestinal muscle.

Our previous study of neural cell adhesion molecule (NCAM) in childhood Hirschsprung's disease demonstrated increased expression on intestinal muscle, especially muscularis mucosae, in aganglionic bowel. The present study was undertaken to test whether this increased NCAM expression is a feature of congenital aganglionosis rather than being a nonspecific feature of constipation. We studied specimens from six patients (20-60 years old; five women and one man) operated on for idiopathic long-standing constipation, using immunocytochemistry for NCAM and protein gene product 9.5 (PGP 9.5). Results showed that in adult constipated bowel the pattern of NCAM expression is similar to that seen in controls. There was no expression of NCAM on muscle in any of the specimens studied. Thus, it seems likely that increased NCAM expression in gastrointestinal smooth muscle in congenital aganglionosis is a result of the abnormal innervation rather than any symptomatic or clinical feature.

Adult↗
PubMed1996

Coping strategies of children with faecal incontinence.

As part of a study concerning the psychosocial adjustment of 160 children treated for anorectal anomalies, the authors investigated the ways in which the children coped with faecal incontinence (FIC). At the time of assessment, the children were 6 to 18 years of age. Portions of the in-depth interviews with the children and their parents covered questions about methods of managing and coping with FIC at home, socially, and at school. In addition, information was obtained about child and family characteristics that have been shown to contribute to the ability to adapt to chronic health problems. The ways in which the children dealt with their problems could be grouped into three distinct phases and were different for boys and girls. In phase 1, around 6 to 7 years of age, boys were largely unaware of the unsocial nature of their condition; the girls were sensitive and withdrawn. In phase 2, between 8 and 11 years of age, boys used overt denial, girls used secretiveness. Phase 3, from around 12 years into adolescence, for both sexes was marked by continued covert denial and eventual acceptance of their disability. The coping strategies reflected a complex interrelationship between characteristics of the child, the family, the social environment, and the unsocial and embarrassing nature of FIC. The findings showed that coping with FIC has potentially severely disruptive implications for the overall development of the child.

Adaptation, Psychological↗
PubMed1996

Long-term effects of gastric transposition in children: a physiological study.

Gastric transposition (GT) has become a reliable alternative operation for oesophageal replacement in children. The aims of this study were to assess the long-term results of the operation and to study the function of the intrathoracic stomach. Current symptoms were assessed using a questionnaire and linear analogue scales. Lung function was measured using spirometry and plethysmography, and the results were corrected for height and expressed as a percentage of the predicted values for normal children. Gastric emptying was assessed using a dual isotope radiolabelled test meal (incorporating solid and liquid phases). Full anthropometric and haematologic data also were collected. The results are expressed as medians and interquartile ranges. Seventeen children were examined at least 5 years after GT; the median age was 9 years. Two children frequently had symptoms during swallowing. Four children had significant diarrhoeal episodes, and two had significant postprandial weakness or dizziness. Unexplained breathlessness was noted by four children. All but one child had lung function values that were lower than the mean predicted value for height. For example, the total lung capacity was 68%, and forced vital capacity (FVC) was 64%. However, the ratio of forced expiratory volume in 1 second (FEV1) to FVC was normal. The gastric emptying study showed that the intrathoracic stomach in all subjects served as a conduit (rather than a reservoir) for both liquids and solids. Rapid emptying (> 50%) in both phases occurred within 5 minutes of ingestion in 82% of the group. Thirteen children were between the 3rd and 97th percentiles for height, and 11 in this range for weight. Five children were anaemic (< 11.5 g/dL). In 11 of the tested samples, the serum ferritin was low, indicating depleted iron stores. GT is compatible with an entirely normal life and has allowed satisfactory growth and nutrition for the majority of subjects in this study group.

Anastomosis, Surgical↗
PubMed1996

Associated urologic anomalies in low imperforate anus are capable of causing significant morbidity: a 15-year experience.

OBJECTIVES: To determine the incidence and morbidity caused by urologic anomalies in patients with low imperforate anus. METHODS: The case records and imaging studies of 95 patients who underwent surgery for low imperforate anus over a 15-year period were retrospectively reviewed. Follow-up ranged from 2 to 18 years (mean 5.4). RESULTS: Genitourinary abnormalities were seen in 29 patients (31%), whereas 10 (11%) had lumbosacral abnormalities. A micturating cystourethrogram was performed in 48 patients, 18 of whom were shown to have vesicoureteric reflux and 2 of whom required surgical correction. Three additional patients required bilateral ureteric reimplantation: 2 had megaureters whereas 1 had bilateral ectopic ureters. Four patients had evidence of chronic renal failure; these included 1 patient with reflux nephropathy, 2 with bilateral dysplastic kidneys, and 1 with neurogenic bladder. The patient with reflux nephropathy underwent renal transplantation at the age of 18 years. Two patients had proximal hypospadias and 4 had undescended testes. A neurogenic bladder was documented in 5 children, 4 of whom had lumbosacral abnormalities. One of these had a spinal lipoma and required excision of the lipoma and untethering of the spinal cord. CONCLUSIONS: We conclude that 30% of patients with low imperforate anus would have associated urologic anomalies, several of which are capable of causing significant morbidity. Thus, all patients with imperforate anus, whether high or low, need to be screened for associated urologic anomalies.

Abnormalities, Multiple↗
PubMed1996

Operative trauma to the genitourinary tract in the treatment of anorectal malformations: 15 years' experience.

OBJECTIVES: To determine the incidence of urologic trauma during surgical correction of anorectal malformations (ARM). METHODS: The records of 228 children with ARM were retrospectively reviewed. RESULTS: Operative trauma to the genitourinary tract was seen in 12 patients, all male. Of 133 patients with a high ARM, 92 underwent an abdominoperineal pull-through and 41 underwent a posterior sagittal anorectoplasty (PSARP); the incidence of urologic trauma was 12% and 0%, respectively. In the subgroup of male infants with a high ARM (99 patients), the incidence of genitourinary trauma was 11%; in those with a low anomaly, the incidence was 1 of 43 (2%). There were 3 urethral tears, 4 urethral strictures, 5 urethral diverticula, 2 vas deferens injuries, and 1 ureteric injury. Nine of the 12 patients do not have any long-term sequelae resulting from the injuries; however, 2 are now infertile and 1 has urinary incontinence. Four patients are additionally compromised because of an absent or nonfunctioning kidney on one side. CONCLUSIONS: In children with ARM, those undergoing a conventional pull-through repair are more likely to have a genitourinary injury than those undergoing the PSARP, and boys with high ARM are at greatest risk.

Anal Canal↗
PubMed1996

Management of the isolated congenital tracheo-oesophageal fistula.

Over a ten-year period sixteen infants with isolated congenital tracheo-oesophageal fistulas (TOF) were treated. All infants were symptomatic from birth although the diagnosis was only established in the neonatal period in eleven cases. Tube oesophagography was the diagnostic investigation of choice. Bronchoscopic cannulation of the fistula greatly assisted division, which was achieved through a cervical incision in all cases. There were two early recurrent fistulas both of which closed spontaneously. Persistent oesophageal symptoms were common. Seven of the patients (43%) experienced dysphagia, three of whom developed oesophageal strictures requiring repeated dilation.

Deglutition Disorders↗
PubMed1996

Choledochal cyst: varied clinical presentations and long-term results of surgery.

The clinical features and long-term outcome of 21 children with choledochal cyst treated over a 31-year-period is reviewed. All 7 infants ( < 1-year-old) presented with jaundice. Only 3 older children (14%) presented with the classical triad of pain, jaundice and an abdominal mass. Other forms of presentation included pancreatitis (n = 8), cholangitis (n = 3), biliary peritonitis (n = 2) and biliary cirrhosis (n = 2). An incidental diagnosis of a choledochal cyst was made in 3 patients one each with bilateral ureteroceles, renal hypoplasia and meningitis with hepatitis A infection. An elevated serum amylase (SA: mean = 1005 U/L) and intraoperative bile amylase (BA: mean = 16,902 U/L) was observed in all 8 children with pancreatitis. Complete excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy was the primary operative procedure in 18 patients. The remaining patients underwent cystoduodenostomy (n = 2) and cystojejunostomy (n = 1). Recurrent cholangitis and stricture formation complicated cyst enterostomies. In comparison cyst excision with Roux-en-Y hepaticojejunostomy gave excellent long-term results with minimal complication.

Abdominal Pain↗
PubMed1996

Familial hiatus hernia and gastro-oesophageal reflux.

This report describes a family in which the father and three children had a sliding hiatus hernia. In each case there was gastro-oesophageal reflux from the neonatal period which proved unresponsive to medical therapy, ultimately requiring anti-reflux surgery. The trait in this family supports an autosomal dominant genetic component to the infant hiatus hernia.

Chromosome Aberrations↗
PubMed1996

In vivo nuclear transport kinetics in Saccharomyces cerevisiae: a role for heat shock protein 70 during targeting and translocation.

The transport of proteins into the nucleus is a receptor-mediated process that is likely to involve between 50-100 gene products, including many that comprise the nuclear pore complex. We have developed an assay in Saccharomyces cerevisiae for the nuclear transport of green fluorescent protein fused to the SV-40 large T antigen nuclear localization signal (NLS-GFP). This assay allows the measurement of relative NLS-GFP nuclear import rates in wild-type and mutant cells under various physiological conditions. Probably the best understood component of the nuclear transport apparatus is Srp1p, the NLS receptor, which binds NLS-cargo in the cytoplasm and accompanies it into the nucleus. When compared to SRP1+ cells, NLS-GFP import rates in temperature-sensitive srp1-31 cells were slower and showed a lower temperature optimum. The in vivo transport defect of the srp1-31 cells was correlated with the purified protein's thermal sensitivity, as assayed by in vitro NLS peptide binding. We show that the kinetics of NLS-directed nuclear transport in wild-type cells is stimulated by the elevated expression of SSA1, which encodes a cytoplasmic heat shock protein 70 (Hsp70). Elevated Hsp70 levels are sufficient to suppress the NLS-GFP import defects in srp1-31 and nup82-3 cells. NUP82 encodes a protein that functions within the nuclear pore complex subsequent to docking. These results provide genetic evidence that Hsp70 acts during both targeting and translocation phases of nuclear transport, possibly as a molecular chaperone to promote the formation and stability of the Srp1p-NLS-cargo complex.

Antigens, Viral, Tumor↗
PubMed1996

Neural cell adhesion molecule (NCAM) expression in nerves and muscle of developing human large bowel.

Most studies of neural cell adhesion molecule (NCAM) in human musculature are devoted to either developing or adult skeletal and cardiac muscle. The aim of this study was to determine the pattern of NCAM expression in the intestinal musculature of the developing human large bowel. In specimens of large bowel from foetuses (gestational age 8-20 weeks), we examined the immunohistochemical localisation of NCAM in parallel to those of alpha-smooth muscle actin and desmin. Within the developing neural complex, NCAM was expressed at all stages investigated. In intestinal muscle at 8 weeks, immunoreactivity for all antisera was restricted to the muscularis propria. The differentiating muscularis mucosae was demonstrated first at 15 weeks by immunostaining for alpha-smooth muscle actin, and this expression was followed by that of NCAM and desmin at 17 and 19 weeks, respectively. At 20 weeks, NCAM immunoreactivity in the external muscle was intense at the inner border of the circular muscle, with its concentration decreasing towards the outer margin of the muscular wall, whereas alpha-smooth muscle actin and desmin were uniformly distributed in all muscle layers. NCAM is expressed by nerves and muscle of developing human large intestine. Its appearance follows a predetermined pattern, which implies its relevance to the differentiation of intestinal muscle layers.

Actins↗