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Biomedical subjects

J W Puntis

Publications and source records attributed to J W Puntis.

At least 19 recordsLinked to original sources
PubMed2000

Children with neurological disorders do not always need fundoplication concomitant with percutaneous endoscopic gastrostomy.

Whether antireflux surgery should be routinely performed at the time of gastrostomy in children with neurological disorders is debatable because of the risk of gastroesophageal reflux. Some argue that these children should be screened for occult gastroesophageal reflux as this will determine the need for fundoplication. This study retrospectively examines outcome in 29 children with neurological disorders who underwent percutaneous endoscopic gastrostomy (PEG) without concomitant fundoplication. Children were included if they had no clinical evidence of severe gastroesophageal reflux before PEG insertion. The median age of children at PEG insertion was 5.6 years (range 1.1 to 18.0). The children were followed for a median of 2.6 years (range 0.4 to 4.9). Insertion of PEG was technically impossible in two children; and an asymptomatic gastrocolic fistula in another child led to subsequent tube removal. Fourteen of the 26 remaining children developed symptomatic gastroesophageal reflux after PEG; five of these showed no reflux on pH monitoring prePEG. Control of symptoms was achieved by medical intervention in 12, but two required fundoplication. Our findings indicate that in the child with neurological disabilities without symptoms indicating severe gastroesophageal reflux, fundoplication is unlikely to be necessary as a consequence of PEG insertion. We conclude that routine investigation for gastroesophageal reflux in the child without severe vomiting can be avoided and the number of antireflux procedures reduced.

Child, Preschool↗
PubMed2000

Management of septicaemic infants during long-term parenteral nutrition.

Young infants, particularly following gastrointestinal surgery, are at high risk of septicaemia during parenteral nutrition. Febrile illness in the absence of focal infection inevitably raises suspicion of central venous catheter sepsis and poses the following dilemma: remove the catheter (which may then prove uninfected) and lose venous access, or leave the catheter and risk clinical deterioration? We examined retrospectively the isolates from blood culture during febrile episodes in 13 children who received long-term (> 2 months) parenteral nutrition via a central venous catheter, and assessed the effectiveness of through-catheter antibiotic treatment during 76 episodes of blood culture positive sepsis. Coagulase-negative Staphylococci accounted for only 16% of positive isolates, with yeasts accounting for 5%, and Gram-negative organisms accounting for 46%, suggesting that infection was often associated with bacterial translocation from the gastrointestinal tract. Treatment with the central venous catheter left in situ was successful in resolving infection in 53 (70%) of septic episodes. These findings indicate that, in this specific group of patients, through-catheter antibiotic treatment is often effective in treating septicaemia. When long-term venous access is essential, this approach should be tried before recourse to central venous catheter removal.

Anti-Bacterial Agents↗
PubMed1999

Limited ileal resection in infancy: the long-term consequences.

BACKGROUND/PURPOSE: The longer-term sequelae of short bowel syndrome in infancy are reasonably well documented, but little is known about the long-term nutritional and metabolic effects of limited (less than 50 cm) ileal resection. This makes it difficult to formulate a rational follow-up policy in such children. METHODS: All children who underwent limited ileal resection for either necrotizing enterocolitis (NEC) or intussusception at our institution between 1984 and 1992 were invited to attend a detailed clinical, anthropometric, hematologic, and biochemical assessment, together with a biliary and renal ultrasound scan and measurement of bone mineral density. RESULTS: Twenty-four children (NEC, 17; intussusception, 7) of median age 7.4 years (range, 5.5 to 13.7 years) agreed to participate. Nine previously had undergone an isolated ileal resection, and 15 also had had variable lengths of colon removed. The length of resected ileum ranged from 3 to 44 cm, with a median of 10 cm. Seven control subjects in whom neonatal NEC developed but recovered without surgery were also evaluated. Median height, weight, and body mass index after ileal resection were between the 25th and 50th percentiles; no child was stunted or wasted. After ileal resection, one boy was found to have asymptomatic vitamin B12 deficiency, and three children had low plasma concentrations of vitamin A. Hematologic and biochemical parameters were otherwise normal apart from a few marginally low trace element levels in both subjects and controls. No renal calculi were detected, and bone mineral density measurements were normal in all except one child. Four children had cholelithiasis, all of whom had previously undergone limited ileal resection for NEC (two isolated, two ileocolic). Thus, the prevalence of cholelithasis after limited ileal resection for NEC was 24% at a median age of 7.0 years. CONCLUSIONS: Growth and nutritional status are well preserved after limited ileal resection in infancy. Limited ileal resection for NEC is associated with a subsequent high prevalence of cholelithiasis and a risk of vitamin B12 deficiency. These findings are important when planning strategies for long-term follow-up.

Bone Density↗
PubMed1999

Cow's milk enteropathy: surgical pitfalls.

BACKGROUND: Cow's milk-induced intestinal bleeding is a well-recognized cause of rectal bleeding in infancy. The authors report on 5 older children who presented with either visible rectal bleeding or profound anemia associated with occult intestinal bleeding secondary to cow's milk enteropathy. METHODS: Five children (3 boys and 2 girls) aged between 20 months and 9 years were referred for further investigation of gastrointestinal bleeding. Two had been investigated previously on multiple occasions, and both had undergone laparotomies with negative results. Further investigations showed evidence of allergic colitis in 3 detected only on proximal colonic biopsy findings. RESULTS: In all cases, bleeding resolved completely after instituting a cow's milk-free diet. Two of the patients subsequently have undergone a cow's milk challenge leading to prompt recurrence of symptoms, which again resolved after simple dietary manipulation. CONCLUSIONS: Cow's milk enteropathy may cause overt rectal bleeding or profound anemia from occult intestinal bleeding even in older children. Histological abnormalities in such cases may be confined to the proximal colon. After appropriate investigation, a trial of cow's milk exclusion should always be considered before laparotomy for obscure, chronic gastrointestinal bleeding in children.

Child, Preschool↗
PubMed1999

Small bowel intussusception in celiac disease.

Intussusception is not a widely recognized complication of celiac disease and yet it is not rare. The authors report on 3 children with spontaneously resolving small bowel intussusception in association with celiac disease. Small bowel intussusception in a child with suspected celiac disease initially should be managed expectantly rather than by early surgical reduction. The finding of transient small bowel intussusception, either by contrast radiology or sonography, should prompt investigation for celiac disease.

Celiac Disease↗
PubMed1999

How often do parenteral nutrition prescriptions for the newborn need to be individualized?

BACKGROUND: Parenteral nutrition is commonly given in the newborn period to premature infants or those with gastrointestinal disorders. Computer-assisted prescribing is widely used, with prescriptions for each patient being varied on a daily basis. It has previously been suggested that 'individualization' of feeds may have little clinical benefit whilst increasing pharmacy workload and costs. However, the scope for use of standard feed solutions as an alternative remains uncertain. METHODS: To assess the potential for using standardized pre-mixed feeds we prospectively reviewed 148 computer assisted prescriptions for newborn infants in order to establish how often the prescribing clinician adhered to the computer protocol, and the reason for modification when this occurred. RESULTS: Only one-fifth of feeds were based strictly on the computer recommendation with no, or minimal, modification. However, many of the deviations in the other four-fifths of feed prescriptions reflected a routine use of higher carbohydrate, sodium and phosphate intakes implying that a higher proportion of feeds could be 'standardized' if the computer regimens were modified to reflect current nutritional practices on the unit. CONCLUSIONS: This study suggests that the introduction of standard PN feeds could considerably reduce the use of computer assisted individualized PN prescriptions on the neonatal unit. The practical implications of such a system for pharmacy and the potential cost benefits deserve further investigation.

Decision Making, Computer-Assisted↗
PubMed1999

A prospective audit of paediatric colonoscopy under general anaesthesia.

Colonoscopy in children is frequently performed using intravenous sedation. Traditionally, there have been few advocates of general anaesthesia and some have regarded colonoscopy conducted in this way as potentially more hazardous. The aim of this study was to undertake a prospective audit of paediatric colonoscopy carried out under general anaesthesia. The details of all children referred for colonoscopy during a 3.5-y period were collected prospectively and the safety and efficacy of performing colonoscopy under general anaesthesia were analysed. A total of 250 colonoscopies was performed in 215 children of median age 10.7 y (range 5 months to 16 y) and ileoscopy was carried out in 164 of these cases. An increasing proportion of patients was investigated as day-cases, including most of the 56 who had additional procedures carried out under the same anaesthetic. There were no complications from the colonoscopy (including the 18 patients who underwent polypectomy). Only one procedure-related complication occurred and this was avoidable. These results confirm the safety of paediatric colonoscopy under general anaesthesia and demonstrate the advantages and feasibility of such an approach.

Adolescent↗
PubMed1998

Overexpression of an unstable intrinsic factor-cobalamin receptor in Imerslund-Gräsbeck syndrome.

Two sisters with Imerslund-Gräsbeck syndrome who presented with clinical features of cobalamin deficiency are described. Intrinsic factor-cobalamin receptor (IFCR) activity and protein levels were determined in ileal biopsy specimens by using radioisotope assay and immunoblotting, respectively. IFCR activities in ileal homogenates expressed as femtomoles of ligand binding per milligram of protein were 38 +/- 4 in control tissue, 494 +/- 24 in patient 1, and 94 +/- 7 in patient 2. However, when assayed in the presence of IFCR antiserum, the ligand binding was inhibited by > 90% in both normal control and the patients with Imerslund-Gräsbeck syndrome. Immunoblotting of total membranes from the biopsy specimen of these 2 patients failed to detect an immunoreactive band of molecular mass of 185 kilodaltons. These findings are at variance with reports of decreased IFCR activity and indicate a new phenotype in which an active but an unstable receptor is overexpressed in Imerslund-Gräsbeck syndrome.

Child↗
PubMed1998

Necrotizing enterocolitis after gastroschisis repair: a preventable complication?

BACKGROUND: Necrotizing enterocolitis (NEC) has been documented in up to 20% of infants after repair of gastroschisis and is responsible for significant morbidity. NEC is reported to occur up to 10 times more in preterm infants receiving standard formula compared with those who have been fed exclusively with breast milk. Does breast milk confer a similar protection against NEC in infants who have undergone surgery for gastroschisis? METHODS: All newborns with gastroschisis delivered between 1990 and 1996 and treated in a single neonatal unit were analyzed retrospectively. Clinical data, details of feeding regimens, and episodes of definite NEC were recorded. RESULTS: Of 60 infants with gastroschisis, 6 (10%) died but none had evidence of NEC. Of the remaining 54 infants, clinical and radiological signs of NEC developed in 8 (15%). All recovered with medical treatment including the three patients with recurrent episodes. NEC developed in none of the 12 babies exclusively fed with expressed breast milk (EBM) in contrast to 1 (5%) of the 19 who received both EBM and formula, and 7 (30%) of the 23 who were fed solely on formula. There was no significant difference in gestation, incidence of primary versus silo closure, or incidence of intestinal atresia/stenosis in those with NEC (n=8) compared with those without (n=46), but birth weight in the NEC group was lower. NEC was less likely to develop in infants who received EBM than those who were exclusively formula fed (P < .02). CONCLUSION: After gastroschisis repair, feeding with maternal expressed breast milk may help to protect the infant from developing NEC.

Abdominal Muscles↗
PubMed1998

The economics of home parenteral nutrition.

During the past 30 y long-term parenteral nutrition has evolved from a novel technique to a well-established intervention for gastrointestinal failure. The development of home care has been driven by technological advances and patient/family choice as well as financial constraints, reflecting a shift in resources from hospital to community. Annual costs for adult home parenteral nutrition have recently been estimated to range from US $150,000-250,000 in the USA, and are around 55,000 Pounds in the UK, perhaps only 25-50% of in-hospital costs. In the absence of any alternative treatment for children with gastrointestinal failure, parenteral nutrition is life saving, and when performed at home offers the prospect of maintaining a good quality of life. Cost-utility appraisals of home parenteral nutrition in adults incorporating quality of life assessment have shown the cost of one quality adjusted life year to be around 69,000 Pounds in the UK (1995), and Can $14,600 in Canada (1984), making home parenteral nutrition relatively cost-effective compared with other ways of spending money to improve health. Because there are no published economic appraisals of pediatric home parenteral nutrition, this paper is based on a review of the literature relating to adult patients.

Adult↗
PubMed1998

Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy.

AIM: To evaluate long term detailed pancreatic endocrine and exocrine function in children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) after 85-95% pancreatectomy. METHODS: Six children with PHHI between 0.9 and 12.7 years after pancreatic resection underwent clinical and investigative follow up at 1.0 to 14.9 years of age. One child with PHHI who had not had pancreatectomy was also assessed. Standard endocrine assessment, pancreatic magnetic resonance imaging (MRI), and detailed direct and indirect tests of exocrine pancreatic function were performed. RESULTS: Pancreozymin-secretin stimulation test results were normal in only one child, borderline in two, and deficient in four, one of whom requires daily pancreatic enzyme supplements. Pancreolauryl tests performed in three children were borderline in two and abnormal in the other. Only one child had low faecal chymotrypsin values. One child developed insulin dependent diabetes at 9 years and two children at 1.0 and 13.3 years require diazoxide to maintain normoglycaemia. MRI showed no major regrowth of the pancreatic remnant after resection (n = 5). CONCLUSIONS: Clinical evidence of endocrine or exocrine dysfunction has developed in only two patients to date, but detailed pancreatic function testing suggests subclinical deficiency in all but one of our patients with PHHI. Although 95% pancreatectomy results in postoperative control of blood glucose, subclinical pancreatic insufficiency is present on long term follow up and development of diabetes mellitus and exocrine failure remain ongoing risks.

Child↗
PubMed1997

Duodenal web presenting with acute pancreatitis.

An 8-year-old boy presented with acute pancreatitis and was found to have a fenestrated duodenal web (windsock) and associated anomalies. After partial excision of the web and duodenoplasty, he has remained well with no further symptoms.

Acute Disease↗