Pancreatic exocrine failure in cystic fibrosis presenting as necrotising enteritis.
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Biomedical subjects
Publications and source records attributed to J W Puntis.
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Two infants receiving total parenteral nutrition via a central venous catheter positioned in the inferior vena cava developed an acute abdomen secondary to extravasation of the infusate. The presence of an associated abdominal mass necessitated a laparotomy in one patient. Both infants recovered completely after the catheter had been removed.
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Pancreatic protease deficiency may be an aetiological factor in enteritis necroticans, a disease sharing some features of necrotising enterocolitis (NEC). Using faecal chymotrypsin measurement we have prospectively studied pancreatic exocrine function in infants at risk of NEC. No significant difference was found comparing those infants who subsequently developed NEC and those who did not.
Signs of infection with a central venous access device in situ raise the possibility of catheter sepsis. We evaluated three tests for diagnosis of infection in infants with suspected catheter sepsis. The acridine orange leucocyte cytospin (AOLC) test was 87% sensitive and 94% specific in the diagnosis of catheter-related sepsis defined by quantitative blood culture. The C-reactive protein and nitroblue tetrazolium tests were not as useful. Using the AOLC results, available in an hour, we now remove fewer catheters on suspicion of sepsis alone.
A sick premature baby who requires intensive care will undergo many uncomfortable procedures. It is now accepted that such babies perceive pain and need adequate analgesia, but little is known about the effects of sedation in these patients. We investigated the use of morphine to provide analgesia and sedation for ventilated preterm babies in a randomised, double-blind, placebo-controlled trial. 41 mechanically ventilated babies who had been treated with surfactant (Curosurf) for hyaline membrane disease were randomly assigned morphine in 5% dextrose (100 micrograms/kg per h for 2 h followed by 25 micrograms/kg per h continuous infusion) or 5% dextrose (placebo). Plasma catecholamine concentrations were measured 1 h after the first dose of surfactant and 24 h later. Blood pressure was measured at study entry and after 6 h. The morphine and placebo groups showed no differences in method of delivery, Apgar scores, birthweight, gestation, or catecholamine concentrations at baseline. Morphine-treated babies showed a significant reduction in adrenaline concentrations during the first 24 h (median change -0.4 [95% CI -1.1 to -0.3] nmol/L p < 0.001), which was not seen in the placebo group (median change 0.2 [-0.6 to 0.6] nmol/L, p = 0.79). There was a non-significant reduction in noradrenaline concentration in the morphine group. Blood pressure showed a slight but non-significant fall (median -4 mm Hg) in morphine-treated babies. The incidence of intraventricular haemorrhage, patent ductus arteriosus, and pneumothorax, the number of ventilator days, and the numbers of deaths did not differ significantly between the groups. Morphine, in the dose regimen we used, is safe and effective in reducing adrenaline concentrations in preterm ventilated babies.
A 7 month old infant with pancreatitis and ascites was managed successfully with subcutaneous octreotide and external drainage of a pseudocyst. An endoscopic retrograde cholangiopancreatographic examination showed no congenital abnormality and was consistent with chronic pancreatitis. Octreotide has a possible therapeutic role in pancreatic ascites.
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Ninety-five premature newborns who had hyaline membrane disease and were struggling against the ventilator were randomised to one of three treatment groups: morphine (group M), pancuronium (group P) or morphine with pancuronium (group M+P). The dose of morphine was 50 micrograms/kg per h but was increased to 100 micrograms/kg per h in group M infants if they continued to struggle. The dosage of pancuronium was 100 micrograms/kg given as required to inhibit spontaneous respiration. Plasma catecholamine levels were measured on entry and at 24 h. Blood pressure and ventilatory requirements were determined on entry and at 6 h. The clinical outcome of the infants was documented. Group M infants (n = 29) showed a significant reduction in noradrenaline levels (median change -2.2 nmols/l (range -47.2 to +7.2 nmols/l), although seven were withdrawn from this group because of failure to settle. Group P (n = 28) and group M+P (n = 38) showed no significant change in noradrenaline levels. Comparison between the groups showed that group M infants had a significant reduction in noradrenaline levels compared with group P. The immediate effects of treatment on blood pressure and ventilatory requirements were similar in the three groups. The clinical outcome did not differ for any of the measured parameters. When adequate sedation is achieved, morphine may reduce the stress of newborn intensive care.
Two children are described with anaemia from ileocolic anastomosic ulceration as a late complication of surgery in the newborn period. The anastomosis was revised in each case but in one child there was early recurrence of ulceration.
After prolonged parenteral nutrition a 12 month old infant died with pulmonary hypertension and granulomatous pulmonary arteritis. A review of necropsy findings in 41 infants who had been fed parenterally showed that two of these also had pulmonary artery granulomata, while none of 32 control patients who died from sudden infant death syndrome had similar findings. Particulate contaminants have been implicated in the pathogenesis of such lesions and these were quantified in amino acid/dextrose solutions and fat emulsions using automated particle counting and optical microscope counting respectively. Parenteral feed infusions compounded for a 3000 g infant according to standard nutritional regimens were found to include approximately 37,000 particles between 2 and 100 microns in size in one day's feed, of which 80% were derived from the fat emulsion. In-line end filtration of intravenous infusions may reduce the risk of particle associated complications. A suitable particle filter is required for use with lipid.
A previously unrecognised complication of aspirating ventricular reservoirs is described. Four infants developed fresh bleeding into the cerebrospinal fluid after reservoir taps; ultrasound confirmed intraventricular blood clot in one case. The technique for aspirating the reservoir may have an important bearing on the incidence of this complication.
The steatocrit was measured in infants with protracted diarrhoea who were receiving intragastric modular feeds. Measurements were made when fat intake was constant to determine steatocrit variability and during increases in fat intake to determine fat tolerance limits. Steatocrit variability was expressed as the range between a subject's lowest and highest steatocrit value. The median between-stool and between-day variabilities were 2% and 11% respectively. Variability was also measured in seven healthy breast fed infants. In the five still displaying appreciable physiological steatorrhoea, the between-day variability (median 8%) and between-stool variability (median 9%) were not significantly different from the protracted diarrhoea group. In the fat tolerance investigations in the protracted diarrhoea group, the steatocrit increased with increases in the module fat content. Fat intake and steatocrit were significantly positively related. A significant negative correlation was seen between steatocrit and weight gain, the latter becoming negligible at steatocrit values around 30%.
Two atypical cases of colitis due to cow's milk protein intolerance (CMPI) are reported, affecting preterm infants. One developed a toxic dilatation of the colon and responded well to a casein hydrolysate based feed. The second presented insidiously and failed to tolerate a casein hydrolysate, but responded well to a chicken-based modular feed.
A questionnaire was administered to 70 families with experience of home enteral nutrition. All but one patient received at least some of their feeding overnight. During 11,041 patient days of home enteral nutrition, no serious complications were seen. Sleep disturbance was common, however, and affected 59 parents and 35 children. A nocturnal cough or 'chestiness' were noted in 10 children suggesting occult gastro-oesophageal reflux. Enteral feeding disposables were not prescribable by general practitioners, and funding for the equipment was inadequate in the hospital and community. The mean time to arrange funding for equipment was eight months (range two weeks to two years). Parental views on home enteral nutrition were positive and none felt that its disadvantages outweighed the benefits. Altogether 35 children were described as being more happy and active. Given adequate preparation and continued support at home, parents can manage home enteral nutrition safely and effectively.