Archives ofDisease in Childhood 1992; 67: 109-114

Home parenteral nutrition in chronicintestinal failure

W M Bisset, P Stapleford, S Long, A Chamberlain, B Sokel, P J Milla

AbstractIn children with severe failure of intestinalfunction, intravenous nutrition is at presentthe only treatment able to maintain adequatenutrition for prolonged periods of time. Overthe last five years we have discharged 10patients home on parenteral nutrition for atotal of 25 patient years and here the outcomeof these children is presented. Of the 10patients, one hasdiscontinued home parenteralnutrition (HPN), seven patients remain well,one patient has recently moved to the USA,and one patient has died after major abdominalsurgery. All children had either normal or anaccelerated rate of growth on HPN anddevelopmentally all have progressed well. Allthe children over 5 years attend normalschools. The major complication of treatmentwas line sepsis with an overall rate of oneepisode in 476 days and a total of nine centrallines (five patients) have required replacementgiving an average line life of 680 days. Forthose children unfortunate enough to sufferfrom severe intestinal failure, HPN is prefer-able to prolonged hospital treatment andoffers the chance of a good quality of life withprolonged survival.

Medical ProfessorialUnit, Hospital for SickChildren, LondonW M BissetP StaplefordS LongA ChamberlainB SokelP J MillaCorrespondence to:Dr W M Bisset,Institute of Child Health,30 Guilford Street,London WC1N 1EH.

Accepted 3 September 1991

In early childhood inadequate nutrition readilyresults in stunting of growth and the develop-ment of multiple nutritional deficiencies. Wherenutritional requirements cannot be met by theenteral route as a consequence of severe gastro-intestinal diseaseand intestinal failure, parenteralnutrition offers a method for maintaining normalgrowth and development.15 As a result ofdevelopments both in the formulation ofparenteral feeding regimens and in the insertionand nursing care of central venous catheters it isnow possible for this to be carried out safely formany years.6However prolonged hospital admission has a

detrimental effect on the development of youngchildren and is very disruptive to family life.Home parenteral nutrition (HPN) has beenavailable in selected units within the UK forover 10 years in adult patients,7-9 but it is onlymore recently with improvements in parenteralnutrition care that HPN has become availablefor younger patients. Provisional studies alreadysuggest that the growth and intellectualdevelopment ofchildren fed at home parenterallyis accelerated after discharge from hospital.'0

In this paper we describe the selection,organisation, and outcome of 10 children whomwe have treated at home with HPN for a total ofover 25 patient years and emphasise thoseaspects peculiar to the UK.

Patients and methodsPATIENTSA total of 10 children (five girls, five boys) havebeen discharged from our unit on HPN over thelast five years. Their ages have varied from 10months to 15 years (mean 4-6 years) and thelength of follow up has ranged from 10 monthsto 4-9 years (mean 2-6 years). This representsover 25 patient years experience of HPN. Twochildren have been discharged abroad, one toItaly and the other to Malta, and their care isshared. The details of the patients are listed intable 1.

SELECTION CRITERIA CONSIDEREDThe suitability of a child for HPN is dependenton both medical and social factors. The severityof the child's underlying intestinal disease willdetermine whether complete or partial HPN isrequired, whether they have large fluid, electro-lyte, and vitamin requirements, and will thusinfluence the complexity of the child's treat-ment at home.While it may be possible for the most able

parents to care for a child with complex medicalproblems on HPN, some parents will be unableto cope with the technical and emotionaldemands of their child's treatment. Thus theparents need to be highly motivated, have astable relationship, have adequate housing, andto be of average or greater intelligence.

VENOUS ACCESSIn all patients Hickman or Broviac Silasticcatheters have been aseptically surgically insertedfor the intravenous delivery of nutrients. Ourpreferred site of insertion is in the right or leftsubclavian vein with the tip of the centralvenous catheter lying in the right atrium withthe catheter tunnelled onto the chest wall. Insome children multiple central venous catheterinsertions has meant that jugular and femoralveins have also been used.6 Single lumen

Table I Details of patients who have received HPN

Patient Primary disease Age at Length ofNo discharge follow up

(years) (years)

1 Pseudo-obstruction 3-3 4-92 Pseudo-obstruction 1-7 4 83 Pseudo-obstruction 15-0 3-64 Congenital enteropathy 1-3 3 55 Short gut 1-3 2-76 Short gut 5-5 0 87 Congenital enteropathy 0-8 1-88 Short gut 6-7 1-79 Congenital enteropathy 1 5 0-810 Immnunodeficiency 7-4 0-8

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Bisset, Stapleford, Long, Chamberlain, Sokel, Milla

catheters are used exclusively with their use

strictly limited to the delivery of nutrients.Blockage of a central venous catheter may

occur at the tip from the deposition of fibrin or

at the hub due to the precipitation of nutrientsor salts from the HPN. Urokinase (5000 U in 3ml) instilled into the catheter for four hours andrepeated on successive days up to a maximum ofthree, will dissolve fibrin, and 70% alcohol willhelp dissolve any lipid deposited within thecatheter. If the catheter is blocked at the hub,debris may be seen or the lumen may not bevisible. Removal of the hub and replacementusing a Broviac/Hickman repair kit will restorepatency to the catheter. When these methodsfail the central venous catheter should bereplaced.

NUTRIENTSIn all cases the nutrients were compounded byBaxter Healthcare (nine in UK, one in Italy).Four of the children who have significantresidual intestinal function received an aminoacid (Synthamin, Baxter)/dextrose mixture as a

single infusion. The remaining six children withvery little residual intestinal function receivedintravenous fat (Intralipid 20%, KabiVitrum) inaddition on two days of the week. In three theIntralipid was formulated in a single bag givinga lipid/amino acid/dextrose mixture, and inthree of the older children the lipid was given as

a bolus infusion before the infusion of the aminoacid/dextrose mixture.The electrolyte content of the intravenous

nutrition was closely matched to the require-ments of the child. Trace elements were addedto the infusions: copper, zinc, chromium, andmanganese (MTE-4, Baxter) along with sodiumselenite 0-02 ,umol/kg/day (KabiVitrum). Inthose patients with some residual absorptivefunction, fat and water soluble vitamins were

given as Ketovite tablets and liquid (Paines andByrne): six tablets and 10 ml per day (doublethe normal dose). In two children with verylittle gut absorptive function, vitamins were

given intravenously as Solvito (KabiVitrum)and Vitalipid (KabiVitrum). The shelf life ofnon-lipid containing nutrient bags is threemonths and for those containing lipid it is onlyone month.The intravenous feeds were given at night

over 12 hours and in those children who were

not on total HPN this was supplemented by oralfeeds during the day. When possible the HPNwas given over five or six nights per week toallow the parents a night free.

SUPPLY OF NUTRIENTSFor the patients living in the UK we haveexclusively used the services of a commercialhomecare company (Unicare Medical ServicesLtd, Harlow, Essex) to supply and delivernutrients and disposables to the child's home.The Maltese child received his HPN by airfreight from the UK and the nutrients for theItalian child were manufactured locally. Theintravenous nutrient solutions were delivered atintervals of two weeks to the child's home along

with the ancillary supplies. Space must beavailable in the family home to store intravenousfeeding solutions, consumables, and the itemsof equipment listed in table 2.

FUNDINGThe intravenous nutrient solutions were pre-scribed by the child's general practitioner on aFP1O form and the cost was therefore borne bythe family practitioner budget. The services ofthe homecare company and the recurring costfor the provision of ancillary supplies have beenmet from the budget of the health authority inwhich the child lives.

TRAINING AND COMMUNITY SUPPORTBefore discharge both parents were instructedby our nutrition nurse in the care of the feedingcatheter and the safe delivery of the nutrients.Training takes two to three weeks during whichtime both parents are resident in the hospital.Nursing staff from the referring hospital andcarers from nursery or primary school were alsoinstructed in the emergency care of the centralline. The specific points taught to the parentsare listed in table 3. A home visit was made byour nutrition nurse to assess the suitability ofthe child's home for HPN. The child should

Table 2 Equipment needed for a child on HPN

Intravenous infusion pump (leasing availability from homecarecompany with 4 hour backup service)

Pump standDressing trolley (folding)Refrigerator (for storage of HPN bags)Intravenous giving setsSterile clampsDressing packsChlorhexidineSyringes and needlesSaline and heparinised saline flushSharps' bins and bin bags (with clinical waste disposal service)Catheter capsStorage shelves (giving sets, dressing packs, etc)Mepore dressings (Molnlycke)Adhesive tapeTreatment area (usually in bedroom)13 amp plug pointsTelephoneAdequate plumbing facilities

Table 3 Points taught to both parents before childdischarged home on HPN

Hand washing techniquePrinciples of asepsis:

Preparation of sterile fieldPutting on sterile glovesDrawing up solutions into syringe

Catheter care:Heparinisation and flushing catheterExit site care

Infusion:Priming of giving setsInitiation/termination of infusion

Care of parenteral nutrientsCare of infusion pump:Pump operationMaintenanceAlarms

Monitoring at home:UrinalysisTemperature taking if feverish

Problem solving:TemperaturesAccidental disconnection of lineBlockage of lineAir in lineBreakage/split catheterWho to contact in an emergency (24 hours)

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Home parenteral nutrition in chronic intestinalfailure

ideally have a bedroom to themselves withadequate space in the house for the storage ofthe ancillary items and nutrient solutions. Aprofessional's meeting was arranged so that thegeneral practitioner, health visitor, socialservices, and other community health care

professionals were fully involved and were

aware of the needs of the child and family once

he or she was home. With four patients minoralterations were required to the family home toaccommodate the HPN. All families in the UKhave successfully applied for an attendanceallowance.

MONITORING OF HPNA summary of the monitoring regime used isshown in table 4. Where possible monitoringwas shared with the local referring hospital.

DEVELOPMENTWe assessed the developmental progress of thechildren at the time of discharge and at home byserial assessment. All tests were carried out bythe same clinical psychologist using tests appro-priate for their age. The Griffiths's mentaldevelopment scale was used in those under 3-8years,11 12 the Wechsler preschool and primaryscale of Intelligence (WPPSI) for those under6-5 years,13 and the Wechsler intelligence scalefor children revised (WISC-R) in the olderchildren.'4 The Griffiths's score is presented as

a general quotient (GQ) and the other scores as

an intelligence quotient (IQ).

STATISTICSA paired t test was used to compare height SDscores and the 95% confidence intervals (CI) are

quoted.

ResultsOf the 10 children who were discharged on

home parenteral nutrition, eight are presendyalive and well, one has moved to the USA after2 7 years of follow up (patient 5), and one

patient has died from the surgical complicationsof her underlying short gut syndrome (patient6). Since discharge from hospital the patientshave progress well with few complications,particularly over the last nine patient years

when there have been no infective or nutritionalcomplications in any of our patients. Of theeight well patients, seven remain on HPN whileone child has been able to discontinue intra-venous nutrition after 3- 5 years ofHPN (patient4).

GROWTH AND NUTRITIONBefore starting HPN all of the patient weightswere within the normal range for age and all butone of the heights were within 2 SD of the meanfor their age. One child who was chronicallymalnourished for many years before startingintravenous feeding (patient 8) had a heightwhich was 5-13 SD below the mean. After thestart ofHPN all of the children have maintainedtheir weight within the normal range and therehas been a significant increase (mean of differ-ences 0-837, 95% CI 153 to 0-143) in their SDscore for height. This effect is most noticeablein the stunted patient where the growth velocityincreased from 4 0 cm/year before starting HPNto 10-4 cm/year after. The growth results are

summarised in fig 1.Nutritional deficiencies occurred in the first

patients discharged (patient 1-5) on HPN, butnow with the improved formulation of feedingregimens and better monitoring we have experi-enced no nutritional complications in our

patients over the last 15 patient years. Patient 1

was discharged on oral vitamin supplements butunfortunately these were not taken by the child,leading to the development of Wernicke'sencephalopathy and in another with short gutsyndrome (patient 5) deterioration in stool

2-

0-4.

§ -l-a,

cn 2-2~.1.3

I 9.4-

.5-

6 2 3

Years on HPNa 5

Figure I The change in height as measured by the SD score,

over the period ofHPN. Height unavailablefor patientdischarged to Italy.

Table 4 Investigations for the monitoring of HPN

Time Investigation

Before discharge All investigations shown below

4-6 Weeks Anthropometry Weight, heightHaematology Full blood countBiochemistry Urea and electrolytes, calcium, magnesium, liver

function tests, urine electrolytesBacteriology (if indicated) Urine culture, line site

3 Months Haematology Ferritin, clottingBiochemistry Copper, zinc, selenium

6 Months Haematology Folate, vitamin B-12Biochemistry Vitamin B-1, B-2, B-6, vitamin A, E, DIf indicated Aluminium, chromium, manganese, essential fatty acidsRadiology Liver ultrasound, chest radiography

1 Year Cardiology EchocardiographyPsychology Developmental assessment

ill

0

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Bisset, Stapleford, Long, Chamberlain, Sokel, Milla

losses resulted in zinc deficiency despite intra-venous supplementation. The first five patientsdischarged developed subclinical seleniumdeficiency because their HPN did not containany supplements.'5 In all cases the deficiencieswere corrected with no long term sequelae forthe children.

BIOCHEMISTRYDespite the fact that some of the children hadlarge stool losses of fluid and electrolyte it waspossible in all cases to maintain a good balance.In one child (patient 6) who lost 1 litre of fluidand 150 mmol of sodium per day from a highjejunostomy, this was compensated for byappropriate additions to the intravenous feed.

It is well recognised that liver disease isfrequently associated with long term intravenousfeeding. In three patients (patients 6, 7, 10) theactivity of aspartate aminotransferase waschronically raised above 100 IU/l (normal range<40 IU/1) and in the remaining patients theactivity was maintained either within or justabove the normal range. Only one of these threepatients (patient 7) has undergone serial liverbiopsy, which has shown evidence of mild non-progressive periportal fibrosis. No patients havehad raised bilirubin concentrations at home,although the patient who died after abdominalsurgery developed a cholecyst-enteral fistula,ascending cholangitis, and jaundice as a pre-terminal event. One infant has developed gallstones (patient 7) and has undergone a chole-cystectomy. A second child (patient 9) hadevidence of 'sludge' in the gall bladder that hassubsequently resolved after increased enteralfood tolerance.'6 17

VENOUS ACCESS, LINE CARE, AND INFECTIONVenous access is always a limiting factor in thedelivery of intravenous feeding and great care isrequired to extend the life of feeding catheters.Due to the care and dedication of the parentsthe average line life is 680 days with a range ofeight to 1760 days.The incidence of line sepsis is difficult to

calculate as some septicaemic episodes mayoriginate from the intestine. In six of the 10children (patients 2, 3, 7, 8, 9, 10) no septicaemicepisodes have occurred since discharge home,while a total of 19 episodes have occurred in theremaining four children (patients 1, 4, 5, 6: onepseudo-obstruction, two short gut, one con-genital enteropathy). In 13 of these episodes anenteric organism was isolated from bloodculture and Staphylococcus epidermidis and astreptococcus were responsible for the remain-ing case. The overall rate of septicaemia was onein 476 days.

Line infections were treated with intravenousantibiotics delivered through the line for 10days. Where the patient became very unwelland/or the infection was not brought undercontrol within 72 hours or recurred after thecessation of antibiotics the feeding catheter wasremoved and electively replaced three to sevendays later. Redness at the entry site of thecatheter to the skin tunnel occurred in most of

the patients and settled, in all but one case(patient 4), with the topical skin application ofchlorhexidine. In the latter case the line wasremoved.'8 19A total of nine lines have been replaced in five

of the patients (patients 1, 3, 4, 5, 6). Five wereremoved because of catheter related sepsis, inone the line was damaged beyond repair, inanother poor scar formation around the cathetercuff led to the line falling out, in one patient aninnominate vein thrombosis precipitatedcatheter removal, and in one patient a cathetercould not be unblocked.20 21 In two patientswith a blockage of the catheter hub, replace-ment of the hub using a Broviac repair kitallowed us to save the feeding catheter withoutresorting to replacement of the central line.

DEVELOPMENTAll of the children were within the normal rangeat the time of discharge, and while on HPNtheir development has progressed normally (fig2). Specific delay in the development of speechwas noted in four of the younger children and inthree this corrected after discharge home. In thefourth patient (patient 4) speech therapy andtreatment for middle ear disease has beenrequired.

EDUCATIONAll seven children of school age attend normalschool. One child with an ileostomy (patient 1)goes to a normal school where extra help isavailable. Another child with speech problems(patient 4) as a consequence of a palatal defectand middle ear disease attends a normal schoolwith a special language unit. A third child(patient 8) who missed prolonged periods ofschooling before starting HPN requires someremedial teaching. Physical contact and gameswith other children were not restricted andsome of the children go swimming with theircentral venous catheter protected by a clearwaterproof film (for example, Tegaderm, 3MHealthcare).22 23 The oldest child has recentlysat his university entrance examinations.

FAMILY HOLIDAYSAll the families have been able to take a holidaywith their child. This has inevitably meanttaking the infusion pump and intravenous

14011301120*

° 110

3 100*

so

80

70~0 i 2 3

Years on HPNi 5

Figure 2 The development progress of the children while onHPN as expressed by theirGQ orIQ. Developmental testsdescribed in text. Incomplete data for patient who died.

JIL a I a 9 -1

12

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Home parenteral nutrition in chronic intestinalfailure

nutrients with them and as a consequence mosthave gone by car and have stayed in self cateringaccommodation. We have, however, been ableto arrange holidays in the USA and Sicily as thecompany that manufactures the HPN hassubsidiaries throughout the world.

ORGANISATIONWith seven of the eight British children theiroriginal general practitioners' (GP) agreed toprescribe the HPN. In one case a single handedGP was not prepared to take responsibility forthe child at home and a new GP was found. Thecost of the HPN solutions was £20 000425 000per year but due to special dispensation fromthe Department of Health the GPs were notpenalised. Difficulties in contacting the budgetholder for community services has led toproblems in arranging HPN. Because the refer-ral hospital and the patient's home may be indifferent health authorities there was oftenconsiderable delay between a request beingmade and an agreement reached. On average ithas taken three months to arrange for thedischarge of a child on HPN, although this hasranged from between one month to discharge achild back to Italy to over 18 months todischarge a child home to a south Londonhealth district which has no communityresources. We have found that most healthauthorities have no provision in their budgetsfor the care of children requiring long termmedical treatment at home and as a consequenceit may take some time for them to find therequired funding. The community funding ofthe infusion pump and consumables cost£3000£5000 per year.

DiscussionOur experience over the last five years wouldsuggest that HPN is a safe and efficient meansof delivering nutrients to children with chronicintestinal failure. The children are able to growand develop normally at home with theirfamilies and apart from their gut disease theyare essentially healthy normal children.Many consider parenteral nutrition as highly

dangerous and regard it as a treatment of lastresort. This view stems from the time whennutrients were compounded by ward staff,venous access was poor, and as a result of poorsterile technique morbidity and mortality fromsepsis were unacceptably high. The advances inknowledge regarding parenteral nutrition andits nursing care means that this is no longer thecase and intravenous feeding should be con-sidered at an early stage for the treatment ofsevere gastrointestinal disease. Although it isgenerally assumed that the medical care of achild requiring intravenous nutrition would bebest carried out by trained staff in hospital, ourstudy and a number of others show quite clearlythat this is not the case and that well trained andsupported parents are more than capable ofperforming this task at home. ' 2 4 5 Appreciablyreduced line sepsis rates and prolonged linesurvival in home patients almost certainly relateto the consistent care the children receive from

highly motivated parents and parallels similarfindings in adult patients at home.'9 It isnoteworthy that in two of our patients lineinfections occurred not at home but during briefhospital admissions for respite care.Our data suggest that simple HPN regimens,

which do not require the addition of intravenoussupplements such as vitamins or trace metals athome, are likely to carry the lowest sepsis rates.This is illustrated by patient 1 who had sixepisodes of line sepsis in the first three years ofHPN, at a time when trace elements were beingadded by her mother to the HPN, and nofurther episodes in the subsequent two yearsafter these additions were made aseptically atthe time of bag manufacture.There was no relationship between the type

of infective organism, the underlying intestinaldisease, and the central venous catheter insertionsite. The appreciable reduction in the incidenceof septicaemia in HPN due to both Grampositive and Gram negative organisms, inpatients in whom there had been no change intheir underlying disease, strongly suggests thatthese infections were acquired from exogenoussources. This therefore casts doubt on thecommonly held belief that septicaemia with anenteric organism, in a patient with a centralvenous catheter, must be due to endogenousspread from an intra-abdominal source.With the improved life of central venous

catheters it is likely that good venous access canbe maintained in these children into adult life.

Hospital provides the young child with a veryartificial environment, a lack of time for play,inconsistencies in handling, and infrequentmixing with children of a similar age and canresult in the development of mild globaldevelopment delay. Although with appropriateintervention it is possible to reduce this to aminimum, it is probably only once the child isreturned to their home that they are able tomeet their full potential.'0 Our study confirmsthis with normal somatic growth paralleled bynormal neurological and intellectual develop-ment occurring after discharge from hospital.

All nutritional deficiencies occurred in thefirst five patients discharged home, and througha better understanding of the nutritional needsof these children we have not diagnosed anymajor deficiencies in the last three years.2"26However most routine biochemistry laboratoriesare unable to monitor all trace metals, watersoluble vitamins, or essential fatty acids and as aconsequence the need to send specimens tospecial reference laboratories is an addedadministrative and financial burden.The parents are aware that the survival of

their child is dependent on their skill indelivering the HPN and many find this a greatburden. Some parents also feel trapped by theneeds of the child but find that when they seekhelp from support agencies there is no under-standing of the medical problems of the child orthe social or emotional needs of the parents.Despite these problems all the parents feel thatcaring for their child at home is very rewardingand infinitely preferable to the child remainingin hospital. However the group of patients wedescribe here is highly selected and where a

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114 Bisset, Stapleford, Long, Chamberlain, Sokel, Milla

child has multiple organ failure or the family ormedical services are unable to implement intra-venous feeding at home, long periods in hospitalmay be unavoidable.The greatest difficulty and cause of delay in

arranging HPN is in agreeing the financialarrangements. In the USA the move toward theearly institution of HPN in children withchronic intestinal failure has been driven largelyby financial considerations and by the realisationthat children requiring parenteral nutritionprogress better at home from a nutritional andpsychological viewpoint than those childrenwho remain in hospital. This is further facilitatedby the many commercial homecare companieswho are able to manufacture the parenteralfeeds and help train and support the parents athome. As the cost of health care in the USA islargely financed through medical insurancethere are real incentives for the treatment ofchronic problems athome rather than in hospital.

In the UK it costs approximately £100 000each year to keep a child in hospital to receiveintravenous nutrition against a cost of £20 000-£30 000 to receive the same treatment at home.Unfortunately, as a result of the budgetaryarrangements within the National HealthService, the small number of homecare com-panies, and the poor level of development ofcommunity services, the incentives to sendthese children home do not exist and as aconsequence the arrangement ofHPN is difficult.The lack of any proper central funding for HPNin either children or adults means that thefinancial burden of funding this form of treat-ment must be borne by the family practitionerbudget and the community budget of the localhealth authority. Unfortunately this is oftenseen as an added burden rather than as a largeoverall financial saving to the health service.

Because of these delays and difficultieschildren with conditions such as the short gutsyndrome who might benefit from a shortperiod (three to six months) ofHPN while theirintestine is adapting, require to remain inhospital at great expense to the health service. Ifarrangements for HPN could be made moreswiftly, many of these patients would benefitfrom an early hospital discharge.We have relied very heavily on the services of

a commercial homecare company to arrange forthe formulation and delivery of the HPN to ourpatients. Where a unit is caring for children onHPN who are all living close to their hospitalthere may be attraction in providing the wholeservice from local resources. We find that withpatients scattered all over southern England,the homecare company is able to maintainconsistent stock levels of supplies, take care ofmuch of the administration, and continues toprovide a service when the patient moves houseor goes on holiday and have even arranged forthe manufacture ofHPN bags in other countrieswhen the children go abroad on holiday.

For children with severe gastrointestinaldisease unfortunate enough to be dependent onintravenous nutrition, HPN offers the prospectof a good quality of life with prolonged survival.

Many children are likely to benefit from thistreatment but until community services becomebetter developed and funding arrangementswithin the health service more flexible it islikely that many children will continue tolanguish in hospital. Our study shows that thistreatment is not only cheaper to the healthservice but offers the child the prospect ofreduced morbidity with normal growth anddevelopment and a quality of life which allowsthem to fulfil their true potential.

1 Gorski AM, Goulet 0, Lamor M, et al. Nutrition parenteralea domicile chez 1'enfant. Bilan de 8 ans d'activite chez 88malades. Arch Fr Pediatr 1989;46:323-9.

2 Amarnath RP, Fleming CR, Perrault J. Home parenteralnutrition in chronic intestinal diseases: its effect on growthand development. J Pediatr Gastroenterol Nutr 1987;6:89-95.

3 Dahlstrom KA, Strandvik B, Kopple J, Ament ME. Nutri-tional status in children receiving home parenteral nutrition.J Pediatr 1985;107:219-24.

4 Pitt HA, Mann LL, Berquist WE, Ament ME, FonkalsrudEW, DenBesten L. Chronic intestinal pseudo-obstruction.Management with total parenteral nutrition and a ventingenterostomy. Arch Surg 1985;120:614-8.

5 Cannon RA, Byrne MJ, Ament ME, Gates B. Homeparenteral nutrition in infants.J Pediatr 1980;96:1098-104.

6 Pokorny WJ, Black CT, McGill CW, Splaingard ML,Harrison GM, Harberg FJ. Central venous catheters inolder children. Am Surg 1987;53:524-7.

7 Stokes MA, Irving MH. Mortality in patients on homeparenteral nutrition. Journal of Parenteral and EnteralNutrition 1989;13:172-5.

8 Ward MWN, Harrison RA, Doyle J, Clark CG. Parenteralnutrition at home. Practitioner 1984;228:831-3.

9 Griffith CDM, Quale AR, Clark RG, Gurnell P. Homeparenteral nutrition in Sheffield 1978-1983. J Coil SurgEdinb 1984;29:335-8.

10 Ralston CW, O'Connor MJ, Ameny M, Berquist W, ParmaleeAH. Somatic growth and developmental functioning inchildren receivingprolonged home total parenteral nutrition.J Pediatr 1984;105:842-6.

11 Griffiths R. The abilities of babies; a study in mental measure-ment. Amersham: Association for Research in Infant andChild Development, 1954.

12 Griffiths R. The abilities of young children; a comprehensivesystem of mental measurement for the first 8 years of life.London: Clinical Development Research Centre, 1970.

13 Wechsler D. Manual of the Wechsler pre-school and primaryscale of intelligence. New York: Psychological Corporation,1967.

14 Wechsler D. Manual for the Wechsler intelligence scale forchildren-revised. New York: Psychological Corporation.1974.

15 Kien CL, Ganther HE. Manifestations of chronic seleniumdeficiency in a child receiving total parenteral nutrition. AmJ7 Clin Nutr 1983;37:319-28.

16 Cohen C, Olsen MM. Pediatric total parenteral nutrition.Liver histopathology. Arch Pathol Lab Med 1981;105:152-6.

17 Roslyn JL, Berquist WE, Pitt HA, et al. Increased risk ofgallstones in children receiving total parenteral nutrition.Pediatrics 1983;71:784-9.

18 Ladefoged K, Efsen F, Christoffersen JK, Jarnum S.Longterm parenteral nutrition. II. Catheter-related com-plications. Scandj Gastroenterol 1981;16:913-9.

19 Rannem T, Ladefoged K, Tvede M, Lorentzen JE, JarnumS. Catheter-related septicaemia in patients receiving homeparenteral nutrition. Scandj Gastroenterol 1986;21:455-60.

20 Franciosi RA, Ellefson RD, Uden U, Drake RM. Suddenunexpected death during central hyperalimentation.Pediatrics 1982;69:305-7.

21 Graham L, Gumbiner CH. Right atrial thrombosis andsuperior vena cava syndrome in a child. Pediatrics 1984;73:225-9.

22 Berry RK, Jorgensen S. Growing with home parenteralnutrition: adjusting to family life and child development.Pediatric Nursing 1988;14:43-5.

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24 Bowyer BA, Fleming CR, Ilstrup D, Nelson J, Reek S,Burnes J. Plasma carnitine levels in patients receiving homeparenteral nutrition. Am J Clin Nutr 1986;43:85-91.

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26 Geggel HS, Ament ME, Heckenlively JR, Martin DA,Kopple JD. Nutritional requirements for taurine in patientsreceiving long-term parenteral nutrition. N Engl J Med1985;312:142-6.

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doi: 10.1136/adc.67.1.109 1992 67: 109-114Arch Dis Child

 W M Bisset, P Stapleford, S Long, et al. intestinal failure.Home parenteral nutrition in chronic

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