Use of two stage keratinocyte-dermal grafting to treat the separation site in conjoined twins.
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Cells, Cultured↗
Biomedical subjects
L Spitz
Publications and source records attributed to L Spitz.
Necrotizing enterocolitis--multisystem organ failure of the newborn?
Evidence of failure of organ system other than the gastrointestinal tract was reviewed in a series of 46 infants treated for necrotizing enterocolitis (NEC) at a regional combined medical and surgical neonatal unit. Respiratory failure was identified in 42 infants (91%), renal failure in 39 (85%), cardiovascular failure in 15 (33%) and hepatic failure in 7 (15%). The number of systems involved correlated with both severity of disease and outcome. Onset of failure in the above systems was closely related to the diagnosis of NEC and 29 cases predated the clinical onset. We conclude that NEC is part of a syndrome of multisystem failure and that this has important implications for the treatment of NEC.
Cytokines↗
A nuclear localization signal within HIV-1 matrix protein that governs infection of non-dividing cells.
Permissiveness of the host cell to productive infection by oncoretroviruses is cell-cycle dependent, and nuclear localization of viral nucleoprotein preintegration complexes will occur only after cells have passed through mitosis. In contrast, establishment of an integrated provirus after infection by the lentivirus HIV-1 is independent of host cell proliferation. The ability of HIV-1 to replicate in non-dividing cells is partly accounted for by the karyophilic properties of the viral preintegration complex which, after virus infection, is actively transported to the host cell nucleus. Here we report that the gag matrix protein of HIV-1 contains a nuclear localization sequence which, when conjugated to a heterologous protein, directs its nuclear import. In addition, HIV-1 mutants containing amino-acid substitutions in this nuclear localization signal integrate and replicate within dividing but not growth-arrested cells, and thus display a phenotype more representative of an oncoretrovirus.
Amino Acid Sequence↗
Increased expression of muscular neural cell adhesion molecule in congenital aganglionosis.
BACKGROUND: Neural cell adhesion molecule (NCAM) is down regulated during morphogenesis and innervation of cardiac and skeletal muscle. In mature muscle, its reexpression over the entire sarcolemma occurs in response to denervation or paralysis of muscle and in some myopathies. No information is available regarding NCAM expression in human enteric muscle either in health or in disease. Our aim was to test whether NCAM is present in nerves and muscle of normal infant bowel and to determine how its expression is altered in congenital aganglionosis. METHODS: Using immunocytochemistry for light microscopy, we compared the pattern of distribution of NCAM in congenitally aganglionic colon with that in colon from age-matched controls. RESULTS: In normal colon, NCAM immunoreactivity was seen in ganglion cells and nerve fibers throughout the gut wall and, more weakly, on the inner border of the circular muscle. In aganglionic bowel, there was a marked increase in NCAM expression in muscle, particularly that of the muscularis mucosac and characteristic hypertrophied nerve bundles of the intermuscular zone and submucosa displayed immunoreactivity for NCAM. CONCLUSIONS: Abnormal expression of NCAM is, thus, a feature of congenital aganglionosis and is likely to be associated with neuromuscular dysfunction within the affected colon.
Cell Adhesion Molecules, Neuronal↗
Problems with intestinal lengthening.
The surgical problems encountered in treating three infants with short-bowel syndrome are reported. The two survivors demonstrate many of the problems associated with the very short-bowel syndrome and both are dependent on parenteral nutrition despite undergoing intestinal lengthening.
Female↗
Recurrent necrotizing enterocolitis.
In the decade 1981 to 1991, 16 infants developed recurrent necrotizing enterocolitis (NEC). They comprised 12 (6%) of 196 neonates referred for further management of NEC and four others referred with major congenital anomalies. Their median gestational age was 32 weeks (range, 27 to 40), median birth weight was 1,260 g (range, 790 to 3,230), and the sex distribution was equal. Recurrent NEC occurred after a median interval of 37 days (range, 11 to 163) from the onset of the initial episode. All but one of the 16 infants were either premature (n = 10) or mature with major congenital anomalies (n = 5). Nine patients had previously undergone surgery for NEC. Medical treatment was successful in 11 patients with recurrent NEC. Two infants died, both of liver failure related to parenteral nutrition; one of whom had suffered four separate episodes of NEC and was found to have a superior mesenteric artery occlusion. There was no consistent association between recurrent NEC and the type or timing of enteral feeds or the anatomical site or method of management of the original attack. The mortality of 12.5% was similar to that for primary NEC.
Child↗
Immunocytochemistry for neuronal markers shows deficiencies in conventional histology in the treatment of Hirschsprung's disease.
Despite technically satisfactory operations, at least 20% of children with Hirschsprung's disease have an unsatisfactory postoperative result. A possible explanation for their symptoms is the retention of ganglionic intestine which has demonstrable abnormalities of the enteric nervous system. The distribution of intestinal neural proteins and peptides in resected colons from patients with Hirschsprung's disease (n = 10) was compared with that in normal controls (n = 5). Immunocytochemistry was performed using antisera against general markers of the enteric nervous system (PGP 9.5, NSE, NFILs, and S-100 protein) and colonic neuropeptides (VIP, GAL, SP, NPY, CGRP, and Met-ENK). The distribution and density of peptide-containing nerve fibers varied greatly from one patient to another and no consistent pattern of neural disturbances could be discerned in aganglionic colon. At the proximal limit of resection, abnormalities of enteric innervation were detected in 8 of 10 studied specimens. Although ganglion cells staining positively for general neuronal markers were present in all cases, normal populations of neural cell bodies immunoreactive for neuropeptides could be found in only 2 specimens. Enlarged submucosal nerve trunks found in the most proximal area of most specimens, displayed immunoreactivity for general nerve markers and VIP, GAL, NPY, and CGRP. The widely practised conventional histopathological assessment of the proximal limit of colonic neural abnormalities may be inadequate.
Calcitonin Gene-Related Peptide↗
Intellectual development at 3 years of age of children who underwent major neonatal surgery.
Thirty full-term infants born with surgically correctable life-threatening abnormalities who underwent emergency neonatal surgery were followed up from birth to 3 years of age. Comparable data were obtained on a matched group of healthy newborn babies. At 3 years the cognitive functioning of children whose condition had been resolved in the early months of life was similar to the controls. Those children who required further medical or surgical treatment were functioning at lower levels than the controls, with language development being most affected. Number of operations, defined as all procedures under general anesthetic, was the medical factor most strongly associated with poorer outcome at 3 years. Family factors--higher maternal IQ and nonmanual social class--were also positively correlated with language development.
Child, Preschool↗
Papillary cystic cancer of the pancreas: diagnostic difficulties.
Pancreatic tumors rarely present in childhood. Diagnostic difficulty and inappropriate treatment may result especially when the lesions have a cystic component. Two cases of papillary cystic carcinoma of the pancreas in teenage girls are presented. Optimal treatment of such lesions in the head of pancreas consists of partial pancreaticoduodenectomy preserving the pylorus when possible.
Adolescent↗
Aortopexy for tracheomalacia in oesophageal anomalies.
Forty-eight patients with repaired congenital oesophageal anomaly underwent aortopexy for significant tracheomalacia between 1980 and 1990. Indications for aortopexy included recurrent apnoea/cyanosis in 31, near fatal episodes in 16, recurrent respiratory distress and infection in 20 and worsening stridor in 15. Gastro-oesophageal reflux was noted in 30 patients, recurrent fistula in 6 and oesophageal stricture in 14. Aortopexy cured near fatal episodes in all patients and resulted in improvement of airway obstruction in 95%. The procedure failed in 2 patients due to unrecognised bronchomalacia and phrenic nerve palsy respectively. Aortopexy is the primary procedure of choice for significant tracheomalacia when associated with near fatal episodes and significant airway obstruction.
Airway Obstruction↗
Chemotherapy effects on hepatoblastoma. A histological study.
The histopathological features of hepatoblastoma in 17 patients treated with preoperative chemotherapy were compared with those in 11 patients not subjected to chemotherapy during the same 11-year period. Tumor necrosis was more extensive in patients receiving preoperative chemotherapy. Two tumors, however, were apparently unaffected by chemotherapy. There was no obvious correlation between the extent of necrosis and the number of courses of chemotherapy. There also seems to be no evidence of preferential ablation of a particular morphological type of tumor. The most notable feature in cases treated with chemotherapy was the extensive presence of osteoid. Osteoid was present in 36% of untreated cases, occupying < 5% of the surface area, compared with 82% in the treated group. In seven cases, osteoid occupied > 40% of the surface area. This finding raises speculation about the role of chemotherapy in the maturation of tumors that have an inherent ability to differentiate. A long-term study is needed to clarify the prognostic significance of mature heterologous elements in hepatoblastoma.
Adolescent↗
Esophageal atresia and tracheoesophageal fistula in children.
The effect of associated congenital abnormalities, particularly cardiac malformations, on the survival rate of infants with esophageal atresia is emphasized in a number of publications. In infants with non-duct-dependent cardiac anomalies, repair of the esophagus takes precedence whereas in duct-dependent lesions, temporary control can usually be achieved with prostaglandin E. In rare instances, a palliative shunt may have to be constructed prior to the esophageal surgery. The role of gastroesophageal reflux and tracheomalacia in the long-term follow-up is also stressed. Controversy regarding the choice of the ideal method of esophageal replacement continues: one publication compares the outcome of colonic interposition with gastric tube esophagoplasty, whereas the results and short-term follow-up of gastric transposition are detailed in another.
Child↗
Operation for gastro-oesophageal reflux associated with severe mental retardation.
One hundred and seventy six children with severe mental retardation underwent a fundoplication for considerable gastro-oesophageal reflux. There were six 'early' (3%) deaths and five 'late' deaths. Major complications developed in 17 (10%) children whereas 86 (49%) had 'minor' complications. A revision operation was required in 27 patients. Overall 142 (81%) children achieved a good result. In spite of the high complication rate and the need for a secondary operation in 15% of the patients, the quality of life for these children and their parents and carers is greatly improved by antireflux surgery.
Age Factors↗
Current results of surgery for achalasia of the cardia.
Several treatment options are available in the management of achalasia of the cardia. Of a recent series of 14 children, 12 were treated by a modified Heller's myotomy combined with a floppy Nissen fundoplication. Symptoms were dramatically improved in nine during a mean follow up period of 3.9 years. Recurrent oesophageal pain was the most resistant symptom and continued to be moderately severe in three patients, two of whom obtained temporary relief by oesophageal balloon dilatation. Two patients treated by pneumatic dilatation alone have residual symptoms. These results support a primary surgical approach to the management of achalasia in children.
Adolescent↗
Prolonged carriage of Clostridium difficile in Hirschsprung's disease.
The role of Clostridium difficile in the aetiology of diarrhoea in children with Hirschsprung's disease was investigated in a prospective longitudinal study. In 64 children with Hirschsprung's disease no significant difference was found in the isolation rate of C difficile in patients with diarrhoea (32%) and without diarrhoea (26%). Comparable isolation rates were found in 47 control children with and without diarrhoea (27% and 16% respectively). The number of strains producing toxin B was similar in the four groups of children. In contrast to the disappearance of C difficile by 12 months of age in the control groups of children, C difficile could be repeatedly isolated from a proportion of children with Hirschsprung's disease over 12 months of age. These findings help to reconcile the existing contradictory reports on the incidence of C difficile in Hirschsprung's disease associated enterocolitis.
Adolescent↗
Surgical management of neonatal necrotising enterocolitis.
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Enterocolitis, Pseudomembranous↗
Electrogenic colonic ion transport in Hirschsprung's disease: reduced secretion to the neural secretagogues acetylcholine and iloprost.
The effects of the abnormal innervation in Hirschsprung's disease on colonic ion transport were examined in vitro using Ussing chambers. The response of the mucosal/submucosal preparations to different secretagogues were investigated in aganglionic and ganglionic rectosigmoid and transverse colon from children with Hirschsprung's disease and compared with normally innervated colon from children with anorectal anomalies. Basal values were similar in aganglionic and ganglionic rectosigmoid colon. Neurally mediated secretion with iloprost (10(-6) M) and acetylcholine (900 and 9 microM) was considerably reduced in aganglionic colon compared with normally innervated ganglionic colon. The ganglionic colon proximal to the aganglionic colon also had a reduced response to acetylcholine despite a normal acetylcholinesterase staining pattern. The responses to Escherichia coli STa enterotoxin (50 MU/ml) and isobutylmethylxanthine (10(-3) M) were similar in ganglionic and aganglionic colon. The response to STa enterotoxin was not changed by the nerve blocking agent tetrodotoxin (10(-6) M). The data show that colonocytes from aganglionic colon are capable of a normal secretory response if stimulated directly by cAMP or cGMP acting secretagogues but secretion in response to neurally mediated secretagogues is impaired. The hypertrophied acetylcholinesterase positive nerve fibres that infiltrate the aganglionic colon are likely to contribute to the reduced secretion to acetylcholine.
1-Methyl-3-isobutylxanthine↗