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Purpose: Medical researchers deal with a large amount of patient data to improve future treatment decisions and come up with new hypotheses. To facilitate working with a large database containing many patients and parameters, we propose a virtual data shelf, displaying the 3D anatomical surface models in an immersive VR environment. Methods: Thereby, different functionalities such as sorting, filtering and finding similar cases are included. To provide an appropriate layout and arrangement of 3D models that optimally supports working with the database, three layouts (flat, curved and spherical) and two distances are evaluated. A broad audience study with 61 participants was conducted to compare the different layouts based on their ease of interaction, to get an overview and to explore single cases. Medical experts additionally evaluated medical use cases. Results: The study revealed that the flat layout with small distance is significantly faster in providing an overview. Applying the virtual data shelf to the medical use case intracranial aneurysms, qualitative expert feedback with two neuroradiologists and two neurosurgeons was gathered. Most of the surgeons preferred the curved and spherical layouts. Conclusion: Our tool combines benefits of two data management metaphors, resulting in an efficient way to work with a large database of 3D models in VR. The evaluation gives insight into benefits of layouts as well as possible use cases in medical research.

Laparoscopic surgery is thought to be associated with a reduced metabolic response compared to open surgery. Oxygen consumption ($\dot{V}{\rm O}_{2}$) and energy metabolism during laparoscopic surgery have not been characterized in children.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. $\dot{V}{\rm O}_{2}$was measured by indirect calorimetry. Core temperature was measured using an esophageal temperature probe.We found a steady increase in $\dot{V}{\rm O}_{2}$during laparoscopy. The increase in $\dot{V}{\rm O}_{2}$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 $\dot{V}{\rm O}_{2}$.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.

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.

Conjoined twins occur 1 in 200 000 live births with females predominating 3:1. The twins are classified according to their most prominent site of union together with the suffix ‘pagus’. The diagnosis can be suspected on prenatal scan as early as 12 weeks gestation. A recent advance is the use of 3-D MRI imaging to provide accurate anatomical details prenatally.Management can be divided into four phases:•prenatal counselling with termination recommended in cases with cardiac fusion;•non-operative treatment in the presence of complex cardiac or neural fusion;•emergency separation when one twin is dead or threatening the survival of the other or when there is a life-threatening surgically correctable congenital abnormality present;•planned separation usually around 3 months if the infants condition is stable.

Although the overall survival rate for infants with oesophageal atresia now exceeds 90%, the condition continues to be responsible for significant morbidity, mainly as a consequence of associated congenital anomalies of which cardiac malformations (29%) are most prominent. A recently revised risk classification comprising very low birth weight (<1500 g) and/or major cardiac defects more accurately predicts survival in the modern era. Early complications following repair include anastomotic leaks, strictures and recurrent fistula, while late complications comprise tracheomalacia causing acute life-threatening attacks, gastrooesophageal reflux, and respiratory and oesophageal motility disorders.

Carnitine palmitoyltransferase I is assumed to be rate limiting for β-oxidation in all tissues. However, the concentration of malonyl-CoA in heart and muscle is high and is enough to completely inhibit β-oxidation if this assumption is correct. In this review, we consider whether: (i) there is a malonyl-CoA-insensitive carnitine palmitoyltransferase I activity; (ii) the measured malonyl-CoA concentration in the heart is physiologically meaningful; and (iii) carnitine palmitoyltransferase I is rate-limiting for β-oxidation in the heart.

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 H"2O"2 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 @mmol/L) and H"2O"2 (1.5 mmol/L). In study D, morphological alterations induced by NO and NO plus H"2O"2 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 @mmol/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 @mmol/L SNAP and 1.5 mmol/L H"2O"2 to hepatocytes caused further inhibition of oxygen consumption compared with either SNAP or H"2O"2 alone. In study D, mild alterations in hepatocyte morphology were noted in the presence of SNAP or SNAP plus H"2O"2. 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 H"2O"2. Morphological findings were consistent with these biochemical effects and suggest that NO and H"2O"2 are important mediators of liver damage during sepsis.

Aims To document the long term course of chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS) in children with defined enteric neuromuscular disease, and the place and type of surgery used in their management; in addition, to identify prognostic factors. Methods Children with CIIPS were investigated and treated prospectively. Results Twenty four children presented congenitally, eight during the 1st year of life, and 10 later. Twenty two had myopathy and 16 neuropathy (11 familial). Malrotation was present in 16 patients, 10 had short small intestine, six had non-hypertrophic pyloric stenosis, and 16 had urinary tract involvement. Thirty two patients needed long term parenteral nutrition (TPN): for less than six months in 19 and for more than six months in 13, 10 of whom are TPN dependent; 14 are now enteral feeding. Prokinetic treatment improved six of 22. Intestinal decompression stomas were used in 36, colostomy relieved symptoms in five of 11, and ileostomy in 16 of 31. A poor outcome (death (14) or TPN dependence (10)) was seen with malrotation (13 of 16), short small bowel (eight of nine), urinary tract involvement (12 of 16), and myopathic histology (15 of 22). Conclusions In CIIPS drugs are not helpful but decompression stomas are. Outcome was poor in 24 of 44 children (15 muscle disorder, 10 nerve disease).

Sacral agenesis is a heterogeneous group of congenital anomalies in which most cases are sporadic but rare familial forms also occur. Although one gene has been mapped to chromosome 7q36 in families with hemisacrum, associated with anorectal atresia and presacral mass, it is clear that the genetic aetiology of these disorders is complex and other genes remain to be discovered. Some years ago, the idea of T (Brachyury) as a candidate gene for sacral agenesis was raised, because tail abnormalities associated with T and the t complex, on mouse chromosome 17, resemble spinal defects seen in man. The recent cloning and mapping of the human T gene prompted us to re-evaluate this idea. T is a transcription factor essential for the normal development of posterior mesodermal structures. Although the sequence and function of T are highly conserved in evolution, our genetic study shows that the coding region of the human gene is highly polymorphic. Three common variable amino acid sites in known functional domains have been identified: Gly356Ser, Asn369Ser, and Gly177Asp. For the latter variant, functional studies have shown that the presence of Asp at residue 177 reduces the stability of T dimer formation. A search for rare mutation of T in 28 selected patients with sacral agenesis/anorectal atresia identified a novel, rare variant in one patient and her mother. This mutation leads to an amino acid change within a conserved activation domain. While the functional significance of this single mutation requires further investigation, we can conclude from our studies that if T has a role in the aetiology of sacral agenesis, its contribution is small in this particular set of patients. However, we cannot exclude a more major role in other forms of sacral defect.

Duplication of the alimentary tract is rare but potentially dangerous. Five of 72 children with alimentary tract duplication treated between 1973 and 1992 died from postoperative complications; a further ten required more than one operation. Heal duplications were the commonest, occurring in 16 patients (22 per cent). Thoracoabdominal duplications were the most complicated and responsible for much of the overall morbidity and mortality. Surgical complications were related to the size and location of the duplication, communication with the gastrointestinal tract or vertebral canal, presence of heterotopic gastric mucosa and involvement of mesenteric vessels. Complete excision of the duplication should be possible in most cases.

Between 1981 and 1993, 41 children were treated for hepatoblastoma. Clinical, radiological and pathological data were reviewed retrospectively, focusing on surgical aspects of treatment and outcome. Fourteen children underwent primary resection of the hepatic tumour. One infant with severe congenital anomalies received only palliative treatment. Of 26 with irresectable disease, pulsed cytotoxic chemotherapy (cisplatin and doxorubicin) enabled subsequent surgical excision in 22 and one child with persistent extensive intrahepatic disease was successfully treated by liver transplantation. Thus, with a policy of selective preoperative chemotherapy, 90 per cent of hepatoblastomas were resectable. There were no perioperative deaths from haemorrhage but one child died from an intraoperative tumour embolus. A total of 28 survivors, 27 of whom are disease-free, were followed for a median of 5 years. The cumulative probability of survival in patients treated with intent to cure was 67 per cent. Analysis of survival data suggested a favourable outcome for those with a pure fetal histological tumour subtype. These results demonstrate significant progress in the treatment of hepatoblastoma.

Clinical features and management of 47 children with Klippel-Trenaunay syndrome treated since 1970 were reviewed. Haemangiomas and soft tissue and/or skeletal hypertrophy were present in all 47 patients; venous varicosities developed in 37 (79 per cent). There was no clinical evidence of macrofistulous arteriovenous communications in any patient. Thromboembolic episodes occurred in five children (11 per cent) and 25 (53 per cent) experienced thrombophlebitis. The Kasabach-Merritt syndrome was observed in 21 (45 per cent) and six (13 per cent) presented with high-output cardiac failure. Other manifestations included haematuria in five (11 per cent), rectal or colonic haemorrhage in six (13 per cent), and vaginal, vulval or penile bleeding in six (13 per cent) children with visceral and pelvic haemangiomas. In 26 patients (55 per cent) symptomatic treatment only was required. Surgery was undertaken in selected cases for complications of the haemangioma, for cosmetic reasons and for chronic venous insufficiency. Only one of four children who underwent resection of varicose veins improved. There was no death, but significant morbidity was associated with the treatment of Kasabach-Merritt syndrome and high-output cardiac failure.

Inflammatory myofibroblastic tumours (inflammatory pseudotumours) occurring at intra-abdominal sites in children have rarely been described. This paper reports three patients with this tumour, two of whom presented with fever, anaemia and an abdominal mass, the third with chronic duodenal obstruction. All had experienced significant weight loss. At operation, each had a large fibrous tumour (7–18 cm in diameter) originating from the transverse mesocolon, small bowel mesentery and duodenum respectively. Intraoperative frozen section histological examination in one patient was misinterpreted as a sarcoma. All the lesions were judged to have been completely excised, but one was ruptured during operation and the patient subsequently developed recurrent tumour nodules. Abdominal inflammatory myofibroblastic tumours are rare. They may be suspected before operation but their clinical, radiological and pathological features may be confused with those of malignancy. Complete excision is necessary to avoid local recurrence.

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.

Bar soap shapes have been classified into two main types and four categories or a total of eight possible variations. Bar soap packages were divided into mass market and specialty types each in single and multipack variations and in turn, each category having different styles. An update summary of bar soap transfers, soap wrappers, and soap cartoners completes the description. Finally, for the present and immediate future, one can assume that high speed lines will gain steady acceptance as the new packaging machines become more popular. Specialty soap packaging machines should be made faster and new models are much needed since hand wrapping or manual and semi-automatic machines are the norm today. New wrapping materials, foam polypropylene, pearlescent coated papers, foil laminates will be used more, and new cartons with special protective inner coatings will be offered more widely. Very close cooperation and development work between the finishing line supplier (specifically the soap press supplier), the packaging machine, and packaging material suppliers and the customer should increase. This long overdue cooperation has been seen lately and more of it will play a key role to an active and progressive bar soap industry.

A second patient is described with the syndrome of hypogammaglobulinaemia and malakoplakia; he too responded dramatically to bethanechol treatment and remains well on it.

A total of 303 infants with esophageal atresia and/or tracheoesophageal fistula were treated over 10 years (1980–1989). The overall survival rate was 86.5%. Associated anomalies were identified in 51.8% of patients, the most common being cardiac malformations which affected 24.4% of infants and was responsible for the majority of deaths. The dramatic increase in survival of infants with esophageal atresia in the past half century is due to improvements in pre- and postoperative management, meticulous operative technique to reduce anastomotic complications, and aggressive treatment of associated congenital anomalies.

Abstract: Bar soap shapes have been classified into two main types and four categories or a total of eight possible variations. Bar soap packages were divided into mass market and specialty types each in single and multipack variations and in turn, each category having different styles. An update summary of bar soap transfers, soap wrappers, and soap cartoners completes the description. Finally, for the present and immediate future, one can assume that high speed lines will gain steady acceptance as the new packaging machines become more popular. Specialty soap packaging machines should be made faster and new models are much needed since hand wrapping or manual and semi-automatic machines are the norm today. New wrapping materials, foam polypropylene, pearlescent coated papers, foil laminates will be used more, and new cartons with special protective inner coatings will be offered more widely. Very close cooperation and development work between the finishing line supplier (specifically the soap press supplier), the packaging machine, and packaging material suppliers and the customer should increase. This long overdue cooperation has been seen lately and more of it will play a key role to an active and progressive bar soap industry.

The conventional radiological techniques for the diagnosis of malrotation are prone to widely varying interpretation and may be both hazardous and impractical in the seriously ill infant. The superior mesenteric vein normally lies on the right ventral aspect of the artery, but is found on the left side in malrotation; this change in orientation can be demonstrated by ultrasound, which can provide a rapid and less invasive indication of midgut malrotation. In 19 of 39 patients with suspected malrotation, an abnormal mesenteric axis was demonstrated, and in each case the diagnosis was confirmed at surgery. Of the 20 children in whom malrotation was refuted by ultrasound, normal rotation was supported by oral contrast studies in 14 and an alternative diagnosis established in 6. A further 20 patients were scanned as a control group, and all were found to have normal vascular anatomy. This study supports the hypothesis that as a sensitive and specific investigation for midgut malrotation, the ultrasonographic appearance of the orientation of the superior mesenteric vessels is a safe and non-invasive alternative to conventional contrast studies.

A prospective trial was undertaken to assess the need for routine gastrostomy in the management of the infant with oesophageal atresia. Over a 34-month period, 51 infants with oesophageal atresia and distal tracheo-oesophageal fistula were assigned to either gastrostomy or transanastomotic tube group. The two groups were similar in terms of birth weight, gestational age, risk group, associated anomalies, and anastomotic tension. There was no difference in the number of anastomotic leaks or strictures. The incidence of significant gastro-oesophageal reflux was 72% (n=18) in the gastrostomy group compared with 30% (n=8) in the transanastomotic tube group. forty-four per cent (n=10) of the former required antireflux surgery compared with 15% (n=4) in the latter group. The conclusion was that routine gastrostomy is unnecessary in the management of infants with oesophageal atresia and may be positively harmful in promoting gastro-oesophageal reflux.

The various options for the management of long-gap oesophageal atresia are discussed. Of 89 infants treated, 27 had isolated atresia, 6 distal atresia with proximal fistula, and 56 atresia with distal fistula. The preferred approach for the former two groups was oesophageal replacement via gastric transposition. Recently, delayed primary anastomosis has been attempted after 6–12 weeks. For wide-gap atresia with distal fistula, primary anastomosis under marked or extreme tension with elective paralysis and mechanical ventilation for 5 days postoperatively achieved highly successful results in 39 infants. There were no major anastomotic disruptions and only 7 minor leaks. Strictures developed in 72% of cases and gastro-oesophageal reflux in 54%, 66% of whom required antireflux surgery. There was only 1 death in the 43 patients undergoing oesophageal replacement (none after gastric transposition, n = 34). A highly satisfactory outcome was achieved in 85%–90% of infants undergoing a replacement procedure.The various options for the management of long-gap oesophageal atresia are discussed. Of 89 infants treated, 27 had isolated atresia, 6 distal atresia with proximal fistula, and 56 atresia with distal fistula. The preferred approach for the former two groups was oesophageal replacement via gastric transposition. Recently, delayed primary anastomosis has been attempted after 6–12 weeks. For wide-gap atresia with distal fistula, primary anastomosis under marked or extreme tension with elective paralysis and mechanical ventilation for 5 days postoperatively achieved highly successful results in 39 infants. There were no major anastomotic disruptions and only 7 minor leaks. Strictures developed in 72% of cases and gastro-oesophageal reflux in 54%, 66% of whom required antireflux surgery. There was only 1 death in the 43 patients undergoing oesophageal replacement (none after gastric transposition, n = 34). A highly satisfactory outcome was achieved in 85%–90% of infants undergoing a replacement procedure.

Nuclear import of the retroviral preintegration complex and integration of retroviral with host cell DNA are essential steps for completion of the virus life cycle. The preintegration complex of the lentivirus human immunodeficiency virus type 1 (HIV-1) displays karyophilic properties and, as a consequence, is rapidly directed to the host cell nucleus by an energy-dependent transport pathway. The karyophilic properties of nuclear proteins are governed by a nuclear localization sequence, the targeting function of which can be inhibited in the presence of excess targeting signals. Here we present evidence that the nuclear import of a large karyophile--the preintegration complex of HIV-1--is inhibited in the presence of a prototypic nuclear targeting signal of simian virus 40 T antigen. This points to a novel strategy which prevents establishment of the provirus by interrupting nuclear localization of HIV-1 DNA.

Two children with sacrococcygeal chordoma are described. In childhood the chordoma is a highly malignant, locally infiltrative, and rapidly fatal tumor. Distant metastases are uncommon. Radiotherapy is the only definitive treatment, although our cases were totally resistant to all forms of therapy.

During the 5 years 1985–1989, 24 (32%) of 76 patients treated for Hirschsprung's disease (HD) developed enterocolitis, this being present at the time of diagnosis in 10 (13%) infants, 7 of whom were neonates. HD presented as necrotizing enterocolitis in 5 neonates, 4 of whom were premature. The enterocolitis developed postoperatively in 14 (18%) patients, in 7 after an enterostomy and in 7 after a pull-through procedure. Recurrent episodes of enterocolitis occurred in 4 of the patients who developed postoperative enterocolitis. The risk of enterocolitis was increased in girls, in patients with associated Down's syndrome, those with a family history of HD, and those managed by an endorectal pull-through procedure. The Duhamel procedure was associated with a low (5%) incidence of postoperative enterocolitis. Long-segment aganglionosis was not associated with an increased risk of enterocolitis and preoperative enterocolitis conferred no increased risk of postoperative enterocolitis.

An audit of 143 consecutive neonatal surgical operations performed in one neonatal surgical unit is reported. Wound infections developed in 13 patients (9.1%) and bacteraemia in 9 (6.3%). Three groups at increased risk were identified: small-for-dates infants subjected to major surgery, infants subjected to invasive monitoring, and infants over 1 week of age with Staphylococcus aureus as a skin commensal. Infections were significantly less frequent in potentially contaminated wounds if antibiotic prophylaxis had been used (7.6% compared to 26.7%: P <0.05).

Children with total colonic aganglionosis pass through three critical phases, the first extending from birth to the establishment of the correct diagnosis with construction of a ganglionic stoma. The second phase lasts from the raising of the stoma to its closure, including the definitive surgical procedure. The third phase begins with closure of the stoma and ends in a gradual decrease in the frequency of defaecation together with the acquisition of continence. The three phases have been analysed in two groups of patients; group I presented between 1978 and 1982 (n = 23) and group II between 1983 and 1987 (n = 17). In the first phase, 9 children in each group underwent inappropriate surgery. The mortality in the second phase was 26% in group I and 6% in group II and in the third phase 6% and 0% respectively. Lack of awareness of the condition and a reluctance to transfer neonates with intestinal obstruction to specialised centres led to delay in diagnosis and inappropriate treatment in both groups. In the second phase, improvements in results were due to increased use of parenteral nutrition, careful monitoring and replacement of stomal electrolyte losses, and improved management of enterocolitis. During the third phase, nocturnal incontinence remained a problem.

Between 1978 and 1986, 55 neonates were treated for small-bowel atresia, stenosis, or segmental dilatation with an overall survival rate of 89%. Before 1981, 21 patients were treated with a mortality rate of 14%, whereas the mortality rate for the 34 treated after 1981 was 9%. Whenever possible, an end-to-end single layer anastomosis was performed with a mortality rate of 6%. In very sick patients with complicated lesions, defunctioning stomas were the safest form of treatment, but patients so treated had a mortality rate of 12.5%. The main causes of death were other severe congenital anomalies.

Eight children ranging in age from 1 day to 6 years with mesenchymal hamartoma of the liver are reported. The diagnosis was established by the characteristic ultrasound finding of a mass of mixed cystic and solid consistency. The treatment of choice is operative resection, which was accomplished with low postoperative morbidity and no mortality. By reducing excessive vascularity, angiographic embolisation facilitated resection in one patient.

This report comprises ten children with hyperinsulinaemic hypoglycaemia who did not respond to medical management and required surgical treatment. Eight were infants under the age of 1 year while two were children aged 3.5 and 5 years, respectively. In each case the diagnosis of nesidioblastosis was confirmed at operation by frozen section histopathological examination and a 95% pancreatectomy was performed. No patient developed recurrent hypoglycaemia, although transient hyperglycaemia, which temporarily required insulin therapy, appeared in five children. All ten patients are now normoglycaemic without treatment.

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.

Normal blood flow was measured in two regions of the ileum (distal and proximal) of normal birth weight (NBW) and low birth weight (LBW) neonatal piglets. Compensatory collateral blood flow in response to occlusion of vessels in the mesenteric vascular arcades was also measured in distal and proximal ileum of NBW and LBW neonatal piglets. Under normal control conditions the blood flow in the distal ileum of NBW piglets is reduced (40% less than proximal) and in LBW animals this reduction is greater (55% less than proximal). Both proximal and distal ileum of NBW animals but the proximal ileum only of LBW animals could mount compensatory collateral flow, whereas the distal ileum of LBW animals was unable to do so. This decompensation in LBW distal ileum may explain the predilection of NEC lesions for the distal ileum and proximal colon.

Occlusion of groups of vessels in the mesenteric vascular arcades of distal ileum for 48 h induced necrotizing enterocolitis lesions in low birth weight, spontaneously delivered, neonatal piglets. Lesion severity increased with numbers of adjacent groups of vessels occluded and with proximity to the ileocecal junction. A previously undescribed feature, "prepneumatosis," was confined to the lymphatic vessels of the submucosa and serosa. This feature closely resembled the position, shape, and distribution of classical pneumatosis intestinalis. Occlusion of vessels for 30 min followed by reperfusion did not induce any detectable changes 48 h later. Identical procedures (48-h occlusions) did not induce any detectable changes in 35-kg pigs.

Abstract Surface electrogastrography was performed in 18 patients with esophageal atresia (EA) and 10 normal controls to investigate the possible role of a congenital enteric nerve defect as a cause of gastroesophageal reflux (GER), which is common after repair of EA. The means of the dominant frequencies and ranges of the frequency distribution were compared. The dominant frequencies (0.047+/-0.007 Hz) in the EA group did not differ significantly from those of the controls (0.050±0.007 Hz,P >0.1), although 2 patients had bradygastria and 2 had tachygastria in the EA group. The range of the frequency distribution was significantly wider in the EA group compared with normal children (P = 0.002). The wide frequency distribution in children with EA suggests disturbed electrical activity of the stomach, which could be associated with poor electromechanical coupling and, hence abnormal gastric contraction.

The manufacture of syndet bars requires special machinery and somewhat different processing steps than those used for normal toilet soap production. Fully synthetic bars are higher priced specialty products which offer special properties not available in normal soaps. Syndet bars: are free from alkali; they can be neutral or adjusted to pH levels below 7; are used for skin problems; lather and clean very well at various water hardnesses without forming a curd or precipitate; are compatible with a large variety of additives; and use less perfume than normal soaps.

Although the use of balloon catheters to dilate oesophageal strictures has been well described in adults (London et al, 1981; Merrell & McCray, 1982; Götberg et al, 1982; Owman & Lunderquist, 1982; Ring & McLean, 1981) its role in children has yet to be evaluated (Pérez-Candela et al, 1983). The technique of balloon dilatation of oesophageal strictures in children as performed on four patients is described.

Gastrointestinal malrotation is recognized to be associated with ventral abdominal wall and diaphragmatic defects. Congenital anomalies in Hirschsprung's disease are generally considered to be fortuitous. The two, when combined form a potentially fatal condition as the clinical and radiological signs and symptoms may be masked. The problems and pitfalls in radiological diagnosis and technique when gastrointestinal malrotation and Hirschsprung's disease occurs simultaneously are discussed.

A total of 303 infants with esophageal atresia and/or tracheoesophageal fistula were treated over 10 years (1980–1989). The overall survival rate was 86.5%. Associated anomalies were identified in 51.8% of patients, the most common being cardiac malformations which affected 24.4% of infants and was responsible for the majority of deaths. The dramatic increase in survival of infants with esophageal atresia in the past half century is due to improvements in pre- and postoperative management, meticulous operative technique to reduce anastomotic complications, and aggressive treatment of associated congenital anomalies. Le pronostic vital des enfants atteints d'atrésie de l'oesophage dépend d'un diagnostic précoce, notamment grâce aux investigations complémentaires poussées, à l-amélioration des soins intensifs périnataux, et à un traitement chirurgical aggressif. En dix ans, 303 enfants porteurs d'une atrésie de l'oseophage et/ou une fistule trachéo-oesophagienne (184 garçons et 119 filles) ont été traités. Seulement 29 (9.4%) avaient une atrésie en l'absence d'air dans le tube digestif sur le cliché de l'abdomen initial. Des anomalies associées, essentiellement cardiaques, étaient présentes chez 157 (51.8%). Selon la classification de Waterson, 96.8% des enfants dans les groupes A et B et 79% des enfants du groupe C1 ont survéçu. Tous les enfants du groupe C2 sont décédés. Une anastomose terminoterminale simple a pu être réalisée chez 226 (77%) des enfants. La morbidité a été de 14% chez les 234 enfants ayant eu un traitement en un temps, alors que des sténoses ont été observées chez 87 (37%) de l'ensemble des enfants traités. La récidive fistuleuse a été constatée chez 17 (6.9%). Quarante-cinq enfants avaient un reflux, dont 23 nécessitant un traitement chirurgical. El año de 1991 marca el 50° aniversario del primer reporte sobre el tratamiento exitoso de una atresia esofágica, atribuído a Cameron Haight en 1941; de lo que anteriormente fue un resultado uniformemente fatal, ahora se espera que la gran mayoría de estos bebés sobreviva. Una pequeña proporción (4–9%) exibe anomalías letales, tales como defectos cromosomiales y síndrome de Potter, y algunos desarrollan hemorragia intraventricular masiva en el período perinatal; la corrección de la lesión esofágica es irrelevante en estos pacientes. El incremento en la tasa de sobrevida en la atresia esofágica puede ser atribuído a varios factores. El diagnóstico precoz ha eliminado virtualmente la neumonía por aspiración; gracias al moderno y sofisticado cuidado neonatal se puede esperar la supervicencia aún de los más niños pequeños (<1.500 gramos); múltiples avances en la atención quirúrgica han venido a reducir la mortalidad y la morbilidad de esta entidad. Hoy es generalmente aceptado que son las anomalías asociadas, especialmente los defectos cardíacos severos, y no la atresia esofágica como tal, la causa principal de muerte. La investigación intensiva y el tratamiento agresivo de estas anomalías deben contribuir a reducir aún más la mortalidad de los recién nacidos con atresia esofágica.

Abstract: The manufacture of syndet bars requires special machinery and somewhat different processing steps than those used for normal toilet soap production. Fully synthetic bars are higher priced specialty products which offer special properties not available in normal soaps. Syndet bars: are free from alkali; they can be neutral or adjusted to pH levels below 7; are used for skin problems; lather and clean very well at various water hardnesses without forming a curd or precipitate; are compatible with a large variety of additives; and use less perfume than normal soaps.

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 (FEV"1) 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.

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.

Abstract: It has been assumed that only 10% of medical interventions are supported by solid scientific evidence. The aim of this study was to determine the type of research evidence supporting operations in a tertiary referral paediatric surgical unit. All patients admitted over a 4-week period to two surgical firms were enrolled in the study. All major operations carried out on each patient since birth were evaluated. Patients for whom a diagnosis was not reached were excluded. A bibliographic database (MEDLINE) was used to search for the articles published between January 1986 and December 1995 on the analysed operations. The type of evidence supporting the operations was classified as follows: I=evidence from randomised controlled trials (RCTs); II=self-evident intervention (obvious effectiveness not requiring RCTs); III=evidence from prospective and/or comparative studies; IV=evidence from follow-up studies and/or retrospective case series; and V=intervention without substantial evidence for or against results of randomised trials. Seventy operations (32 individual types) were performed on 49 patients (1–5 operations/patient); 18 (26%) were supported by RCTs (type of evidence I). Two patients (3%) received a self-evident intervention (type II); 48 operations (68%) were based on non-randomised prospective or retrospective studies (type III=13%; type IV=55%). Two patients (3%) received an operation not supported by or against convincing scientific evidence (type V). A significant proportion of operations in paediatric surgery is supported by RCTs. However, the vast majority of these trials were conducted on adult patients. Sixty-eight per cent of the operations were based on prospective follow-up studies or retrospective case series, which may not represent solid scientific evidence. More RCTs are needed in paediatric surgery.

Nine children (mean age 20 months), with proven primary malignant hepatic tumors have been examined prospectively by high-field magnetic resonance (MR) imaging to assess tumor resectability. All patients had comparative ultrasonography (US), 8 patients had X-Ray computed tomography (CT), and surgical correlation was available in 8 patients. The hepatic and portal veins and the inferior vena cava were visualized in all patients on MR and in 4 of 8 patients on CT. MR accurately defined liver parenchymal involvement in all 8 patients who had surgical exploration. CT underestimated disease in 2 cases, and defined tumour margins less clearly than MR. MR identified abnormal extrahepatic tissue when present, but was unable to distinguish viable tumor from necrotic tumor or reactive nodes. High quality short TR/short TE spin echo images were obtained by combining cardiac triggering and signal averaging. Short TI inversion recovery images demonstrated tumor and lymphadenopathy most clearly. We conclude that MR is the imaging method of choice for the assessment of liver tumor resectability in children.

Research into the pathogenesis of neonatal necrotizing enterocolitis has been a diverse mixture of clinical case reviews, searches for a single cause, analysis of subjective observations and some designed experimental studies. The experimental research has been hampered by the lack of reproducible and clinically appropriate animal models in which to test hypotheses of pathogenic pathways. Reviewing the models which have been evaluated reveals many design inconsistencies and little in depth assessment of the appropriateness of many of these experimental systems. Many species have been studied as potential, clinically adequate models but often without relevant attention to the physiological differences and the effect of age. The piglet seems to present the most extrapolatable data from an appropriate model system for the study of neonatal necrotizing enterocolitis.

Inflammatory mediators such as macrophage-derived cytokines, tumour necrosis factor and interleukins, platelet-activating factor, oxygen-derived free radicals, leukotrienes and complement play an important role in the pathophysiology of multisystem organ failure (MSOF). The similarity in clinical presentation between MSOF and necrotizing enterocolitis (NEC) is considered and the evidence for a possible role of these inflammatory mediators in NEC explored.