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`
`Archives offliu-are in Cltilalhuad 1992; 61': l33T—l 391
`
`I337
`
`PERSONAL PRACTICE
`
`Intercurrent illness in inborn errors of intermediary
`metabolism
`
`Marjorie A Dixon, I V Leonard
`
`Abstract
`in
`Metabolic decolnpensation may occur
`patients with disorders of intermediary meta-
`bolism during intercurrenl illness. To prevent
`complications it is normal practice to change
`the diet
`to an ‘emergency regimen‘. The
`mainstay ofthis is a high carbohydrate intake,
`using soluble glucose polymer, given as
`frequent drinks by day and during the night.
`Additional therapy is given for some disorders.
`Practical details of the treatment are outlined.
`
`(Arch Dis Child l9'92',6T:l38'.'r'-9])
`
`is the mainstay of treatment for many
`Diet
`inborn errors of intermediary rnetabolism.'
`Patients are stabilised on a diet appropriate for
`l.hei.l' disorder
`that will provide them with
`sufficient nutrients and energy to grow and
`develop normally. However metabolic stress
`such as intercurrent infections may precipitate
`decompensation and cause complications. To
`prevent these it is common for the diet to be
`changed to an ‘emergency regimen’. The
`purpose of this paper is to outline the principles
`and practice of these regimens.
`
`Principles
`During illness and fasting various metabolic
`adaptations occur.’
`
`(A) PROTEIN FATMIOLISM
`There is constant turnover of tissue protein and
`overall,
`the rate of protein synthesis exceeds
`that of breakdown so there is net gain and hence
`growth? However. during illness the rate of
`protein breakdown normally exceeds that of
`synthesis with net production of amino acids
`and an increase in their irreversible catabolism.
`The nitrogen moiety of amino acids is converted
`to ammonia and then to urea. The carbon
`skeleton of amino acids is progressively cam»
`bolised to form fuels for energy production
`including acetyl COA, pyruvate, and inter-
`mediates of the Krebs cycle.
`
`(B) suosrmrrcs FOR ENERGY l’lv‘.0DU(.'l'l0N
`Glucose is a major fuel for energy metabolism
`but glycogen reserves for glucose production
`during fasting are relatively limited in children.
`To maintain a supply of substrate for energy
`production and protect glucose supply to the
`
`is necessary to mobilise alternative
`brain it
`fuels, including free fatty acids, ketones, and
`gluconeogenic precursors.
`On fasting, as glucose concentrations slowly
`fall, there is a decrease in the insulimglucagon
`ratio with mobilisation of fatty acids from
`adipose tissue stores. Free fatty acids can be
`utilised by many tissues, such as the heart and
`skeletal muscle, but they cannot enter the brain.
`In the liver,
`free fatty acids are partially
`oxidised to ketones which are water soluble and
`can enter the central nervous system. Alanine
`and other amino acids from muscle catabolism
`and glycerol from lipolysis are substrates for
`gluconeogencsis.
`The purpose of the emergency regimen is to
`prevent
`the changes that occur with fasting.
`The aim is to reduce protein catabolism and
`hence the accumulation of potentially toxic
`metabolites. By giving an adequate supply of
`glucose the mobilisation of alternative fuels is
`also reduced.
`
`Practical aspects of emergency regimens
`The core of the emergency regimen is csscntially
`similar for all disorders. A solution of glucose
`polymer is given as the major source of energy
`because it is simple, palatable, and usually well
`tolerated. Fat emulsions can provide additional
`energy, but these are less well
`tolerated; fat
`delays gastric emptying and is more likely to
`cause vomiting so we do not use them routinely.
`ll
`is also contraindicated in some disorders,
`such as i.I1born errors of fatty acid oxidation. In
`most instances we would start by giving feeds
`orally. There are important advantages to oral
`feeding. It can be started at home; more glucose
`can be given than by peripheral
`intravenous
`infusions and medicines can be given. This can
`be important as intravenous preparations of the
`medicine are often not readily available. If oral
`feeding is not possible, nasogastric feeding,
`either bolus or continuous, should be tried
`before intravenous therapy for the same reasons.
`The concentrations and volumes of solution
`given will depend on the age of the child.
`Relatively higher volumes and lower concentra-
`tions are used in infants compared with older
`children (table).
`Insufficient fluid combined
`with high concentrations of glucose polymer
`that can cause diarrhoea may exacerbate the
`effects of illness. If the child is likely to become
`dehydrated it is advisable to give an oral re-
`hydration solution supplemented with glucose
`
`LUPIN EX. 1009
`
`1of6
`
`The Hospital lon-
`Sich Children and
`The Institute of
`Child Health,
`London
`Marjorie A Dixon
`J V Leonard
`Utmrrespiutslcnce lo:
`Profaswrl V Leonard,
`Medical Unit.
`Institute of Child Health,
`30 Guilford Srreel.
`lmndon WCIN lE.H_
`
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`
`Ear-rrgmcy regimen
`Age
`Giumre polymer
`{wars}
`nmrnmattian
`$6:
`km-ltltifl in!‘
`
`[LI
`I-2
`2-6
`6—|(l
`zv ll}
`
`[0
`I5
`20
`20
`25
`
`40
`60
`Iii}
`RD
`I00
`
`‘'1 kcaI=-1' Ill
`
`lc].
`
`Daily! wlwne
`
`Frequency
`
`I 511-200 mIIltg
`95 tI'Ili"k
`l2lK|—l
`ml
`|fill)—2lIIl(lml
`2000 ml
`
`Initially every
`two hours
`night and rlay
`
`polymer to the required concentration. (Practice
`note: oral rehydralion solutions do not contain
`sufficient glucose on their own.) The solution is
`given orally as small frequent drinks or enterally
`via a nasogastric tube, normally at intervals of
`two hours night and day. This is usually well
`tolerated. It is particularly important not
`to
`allow the child to go for long periods (>4 hours)
`between drinks during the night.
`Parents are taught to make solutions using an
`appropriate sized scoop which is quick and
`easy. These solutions can also be frozen and
`thawed when needed at home or in hospital. All
`proprietary liquid glucose polymer solutions
`have a concentration of 50% carbohydrate or
`more, for example, Fortical (Cow and Cate) and
`Liquid Maxijul (Scientific Hospital Supplies),
`and are not suitable for an emergency regimen
`unless diluted to an appropriate concentration.
`In our experience these are less palatable and
`more likely to induce vomiting or diarrhoea if
`given undiluted.
`In me children tlte lirst symptom of any
`illness is refusal to eat or drink. if so, we often
`teach the parents how to use a nasogastric tube
`at home,
`thereby reducing the need for a
`hospital admission.
`If the child has occasional vornits, it may still
`be possible to feed orally by giving the drinks as
`frequent sips (for example 10 ml every 10
`minutes) or as a continuous nasogastric feed
`either at home or in hospital. However, if the
`child is vomiting frequently or is obviously
`unwell
`then intravenous therapy is essential.
`Concentrated glucose solutions should be used:
`10% dextrose by peripheral drip or more
`concentrated through a central
`line. Hyper-
`glycaemia may develop so blood glucose con-
`centrations should be monitored regularly.
`
`Instructions for parents
`Many parents have difliculty in knowing both
`when to start
`the emergency regimen and
`exactly what to do. To overcome this it is our
`practice to teach a three stage approach.
`(I) If the parents are uncertain whether their
`child is unwell
`(or
`iust
`tired) because they
`appear lethargic, in-itable, or off colour then
`an emergency regimen drink should be given.
`Next, a Conscious decision is made to reassess
`the child within one to four hours, depending
`on the age and disorder. In some children it is
`possible to monitor the disorder by using simple
`tests at home. These are discussed later under
`the individual disorders.
`(ZA) If on review the child has improved, the
`normal diet is resumed. (B) If on reassessment
`there is no improvement
`the full emergency
`
`Dixa-n,Lemard
`
`regimen of drinks every two hours should be
`started. There is some flexibility in the frequency
`of the drinks particularly in the older children
`and during recovery. During illness most
`children will automatically stop eating the
`normal diet, and once the child starts to
`improve the usual diet is gradually reintroduced.
`(3) If the child is refusing to take the drinks, is
`vomiting frequently, or becoming encephalo-
`pathic then they should be admitted to hospital
`for assessment. The parents need to be aware of
`and recognise clinical signs of deterioration. Of
`particular importance is the ability to recognise
`encephalopathy with the child becoming less
`responsive, often with a glazed look.
`Parents may face difiiculties if the child does
`not take all the recommended volumes of feed.
`The quantities necessary vary both with the
`underlying inborn error and the intercurrcnt
`illness. It is particularly important that patients
`with maple syrup urine disease and methyl-
`malonic acidaemia should have close to the
`recommended volumes (see below). By contrast
`those with glycogen storage disease Inay be
`controlled satisfactorily with smaller volumes.
`Patients with gastroenteritis will in general need
`more fluid than those with upper respiratory
`tract infections.
`It should be emphasised that the basic emer-
`gency regimen must not be continued for long
`periods of time because it does not provide
`adequate nutrition and will cause nutritional
`deficiencies. Early clinical signs of such defi-
`ciencies include skin rashes (particularly at the
`site of adhesive tape for nasogastric tubes).
`Most patients can be gradually returned to their
`usual diet within a few days.
`When the diet is being reintroduced addi-
`tional high carbohydrate drinks are given until
`the normal diet is re-established.
`In infants,
`additional glucose polymer
`is added to the
`formula food. For patients on low protein diets
`the protein intake is usually increased daily,
`giving one quarter, one half, three quarters of the
`usual
`intake,
`resuming the normal protein
`allowance by day four.
`Whenever the child has been ill it is usual for
`us to discuss the emergency regimen carefully,
`checking that the parents knew what to do and
`that the emergency regimen meets the needs of
`their child. It is important to recognise that the
`course of these disorders is often unpredictable.
`The children may have a serious infection
`without any problems, but then develop severe
`de-compensation after apparently minimal stress;
`therefore the need for careful management of all
`intercurrent illness.
`
`Specific treatment
`This basic emergency regimen is suitable for
`disorders of carbohydrate metabolism including
`glycogen storage diseases, fructose-l, 6-diphos-
`phatase deficiency, and ‘ketotic hypoglycaemia‘.
`However,
`in other metabolic disorders the
`emergency regimen is combined with specific
`treatment.
`
`tvmrm svnur URINE Dist-msr: (Msun)
`In MSUD the branched chain keluacid de-
`hydrogcnase is defective. This enzyme is the
`
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`II-ztercirrrent illness in inborn errors of intermediary numirofistn
`
`I389
`
`amino acids should be given.5 Our aim is to
`provide the full energy requirement and the
`child’s usual quantity of BCAA free supplement.
`This is not always possible orally, particularly if
`[he child is vomiting.
`.‘\ useful alternative is
`continuous nasogastric feeding of Maxam-aid
`MSUD (Scientific Hospital Supplies,
`see
`appendix) supplemented with extra glucose
`polymer. We have succcssfiilly used this to treat
`patients with MSUD who have been unable to
`tolerate bolus feeds. Parenteral nutrition, using
`a BCAA free arn.ino acid solution, is an alterna-
`tive if enteral feeding fails but this is available
`in few centres. Regular daily quantitative
`measurements of the BCAA are essential
`to
`monitor progress and to determine when pro-
`tein can be reintroduced.
`Once the plasma leucine concentration falls
`below 800 pmolfl
`some leucine usually as
`natural protein can be reintroduced, and is
`increased to the usual intake according to the
`plasma concentrations. Our aim is to keep
`plasma lcucinc between 200-700 urnolfl. During
`the recovery phase concentrations of the iso-
`lencine and valine may fall to low concentrations
`and become rate limiting for protein synthesis.
`Supplementation of fltese amino acids then
`becomes necessary, in doses of 50-300 mgiday.
`To do this a solution of the amino acids is made
`{providing 100 mg of amino acid in [0 ml) and
`the required amount added to the feed. We do
`not routinely give valine and isolcucine supple-
`ments’ as not all patients need them and this
`practice could cause imbalance in amino acid
`concentrations. Hypoltalaemia may develop
`during the recovery phase.
`
`FHENYLKETONURM (rim)
`Many centres do not give patients with PKU an
`emergency regimen or monitor plasma pheny-
`lalauine concentrations during illness. However
`with increasing emphasis on improving meta-
`bolic control, an emergency regimen is likely to
`be necessary- It should be similar to that used in
`MSUD.
`
`UREA CYCLE nisonoltas
`The urea cycle converts waste nitrogen into
`urea, via a series of enzymes (figure). Inborn
`errors have been identified for each step and
`may cause accumulation of ammonia and gluta-
`mine which are neurotoxic and may cause a
`severe encephalopathy.
`Patients are treated with a low protein diet
`and medicines which promote nitrogen excretion
`via alternative pathways. Sodium benzoate is
`most widely used. It is conjugated with glycine
`to form hippurate,
`l mole of nitrogen being
`excreted for each mole of sodium benzoate
`given. Phenylbutyric acid can also be given
`either as the free acid or the sodium salt but is
`less palatable. Phenylbutyrate is metabolised in
`vivo to phenylaeetate, and is more effective than
`benzoate because it is conjugated with glutamine
`to form phenylacetylglutnmine, 2 moles of
`nitrogen being excreted for each mole of
`phcnylbutyrate given.
`During illness protein breakdown may cause
`
`3of6
`
`second step on the catabolic pathway of die
`three branch chain amino acids (BCAA): leucine,
`isoleucine, and valine. This pathway is the
`major route for dis
`sal of the BCAA. As it is
`blocked in MSU
`the BCAA and respective
`kctoacids accumulate and are responsible for
`the encephalopathy. Losses of BCAA and
`ketoacids in the urine and through other
`pathways are low‘ so the only way to reduce
`plasma concentrations is for the BCAA to be
`incorporated into protein. The obiective of
`treatment during illness is to increase the rate of
`protein synthesis by giving a high energy intake
`as glucose with or without fat. Although the rate
`of protein synthesis may increase initially the
`concentration of the essential amino acids other
`than the BCAA will soon become rate limiting.
`To prevent this supplements of the BCAA free
`
`HEPATIC NITROGEN
`POOL
`Alanine
`Asparrate
`Glutamina
`Glutamate
`Glycine
`and
`other compounds
`
`Phenvlbutvrate
`‘
`Phenylacatata
`
`Benzoata
`
`Phanylacetyl glutamine
`
`AMMONIA
`
`”‘PF'“”"9
`
`16)
`
`CAFIBAMYLPHOSPHATE
`
`ORNITHINE
`
`CITRU LLINE
`
`
`
`UREA
`
`®
`
`AHGININE
`
`AHGINOSUCCINATE G)
`
`Fumarata
`
`URINE
`
`Hepatic nitrogen metabolism. Umt. the major product ofnitrogen mutt‘.-o.l't'.rm. is synthesised
`ututltitr tire urea r.yc.lefi'a.m
`note and nlmnmia. Defects ofeacit step in the cycle from been
`identified‘. { I } Cnroanryipimsptmte syruhzture deficiency. (2) Urnitlrrlne curbamyttransferase
`defcie
`. (3)Arginasua1'nates_-yntkctascde ci¢Irt)r(citruJ't'irrne1rria).(-H11 tnosrtccinatr
`lyase de
`ism)! (arginosautinic acidrtrfa). (
`)i‘lI'gr'na.re deficiency. In there
`isorders sodimn
`benaatm and sodium phrgdocetate tarpbemlfbugnmte) are used to reduce theflwr M the urea
`trycle. Benamte is amjngated unit: the gnfitine mfomt kipparate and plteny.l'.err.etare conjugated
`uritir g£uta'mim.1tofwtn plum;-£acc:_v.l giutorniiw. Bad: oftheae compounds are rapidlv excreted
`in the urine reducing the nccumrdarion of mnmomln and amino acids.
`
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`Dixon, Lennard
`
`is to reduce the
`the aim of treatment
`but
`accumulation of propionate by diminishing
`production and increasing disposal.
`In addition to the-standard emergency regimen
`to reduce protein breakdown and amino acid
`catabolism, propionate production from the gut
`is
`reduced by giving metrorlidazole (or an
`alternative antibiotic). The removal of propionyl
`groups is enhanced by giving L-carnitine. This
`forms propionyl camitinc which is excreted in
`the urine. Carnitine is given either orally or
`intravenously in a dose of
`I00 mgfkgfday.
`Higher doses have been used although their
`value and complications have not been assessed
`critically. In methylmalonic acidaemia there is
`usually massive excretion of methylmalonate
`in the urine with obligatory simultaneous losses
`of sodium and potassium. These patients also
`have a concentrating and acidification defect in
`the kidney" so it is essential to give sufficient
`fluid with supplements of sodium bicarbonate
`(2-3 rnmolfkg)
`to replace sodium loss and
`reduce the acidosis. Patients may also need
`potassium supplements particularly during the
`recovery phase.
`It may be helpful to monitor the patient’s
`condition by measuring urine ketones but in our
`experience the parents’ clinical iudgment is as
`useful for most children.
`It is important to reintroduce protein early
`(within two to three days) to prevent protein
`deficiency and additional problems such as
`rashes and vomiting.
`
`(b) Isoealeric acidaemia
`Isovaleric acidaemia is due to a deficiency of
`isovaleryl—CoA dehydrogenase, the third step in
`the pathway of leucine degradation. Isovalerit.‘
`acid accumulates and its excretion in the urine
`can be increased by giving L-carnitine (100 mg!
`kglday) and glycine (250 mg/kgfday). These are
`coniugated to form isovalerylcarnitine and iso-
`valerylglycine respectively, both being rapidly
`excreted in the urine. This treatment can be
`given orally or intravenously.
`
`(for example,
`acidaenzias
`(as) Other organic
`3-ntethylrmtmiylglycinurio, gfntarir ncidnria clips
`
`0 I
`
`n addition to the standard emergency regimen,
`L-carnitine is widely used to increase the
`removal of acyl groups, although controlled
`studies of its efficacy are lacking.
`
`DJSORDERS Oi’ FATTY .:\ClD UXIIMTIDN
`
`Inborn errors at several steps in the pathway of
`fatty acid oxidation are now well described, the
`most common is
`tnedium chain acyl COA
`dehydrogenase deficiency. To prevent illness in
`these patients the standard emergency regimen
`is used. It is important to stress early use of the
`emergency regimen to inhibit mobilisation of
`fatty acids, particularly in long chain disorders
`because decompensatiun may be rapid. Addi-
`tional treatment with Lcamitine is widely used
`but is somewhat controversial;
`there is little
`detailed critical work. Carnitine is essential in
`
`4of6
`
`1390
`
`rapid accumulation of ammonia and glutamine.
`In addition to the basic emergency regimen the
`usual medication should continue to be given.
`Both sodium benzoate and phenylbulyrate are
`given in a dose of 250 mg/kglday but can be
`temporarily increased to 500 mgfkg/day during
`illness.
`Another consequence of these disorders is
`that arginine becomes an essential amino acid
`(except in arginaae deficiency). During ill
`the nonnal arginine supplements, 100 mgfkg!
`day, should be given.
`In citrullinaemia and
`atgininosuccinic aciduria, argininc should be
`given in larger doses of up to 700 tngfkgfday to
`replenish ornithine that is not reformed as a
`result of the metabolic block. All the medication
`should be given in frequent small doses. If the
`child cannot tolerate oral fluids or medicines
`sodium benzoate, sodium phenylaoetate, and
`arginine should be given intravenously.
`Patients should be monitored with regular
`measurement of plasma ammonia and quantita-
`tive plasma atnino acids. Protein can be reintro-
`duced once rhe ammonia is less than 80 utnolrl.
`Occasionally it can be difficult to reintroduce
`protein without
`inducing hyperannnonaemia
`and bi
`these cases an essential amino acid
`supplement (for example Dialamine, Scientific
`Hospital Supplies, see appendix), can be given.
`This will promote both protein synthesis and
`the reutilisation of nitrogen to form non-
`wcenrial an1ino acids thereby reducing ammonia
`accumulation.
`
`TYROSINAEMIA TYPE I (FUMARYLACETOACETASE
`net-'tcIENcv)
`Tyrosinaemia type I is caused by a deficiency of
`fumarylacetoaoetase,
`the last enzyrne of
`the
`catabolic pathway of tyrosine, with resultant
`accumulation of succinylaoetone and related
`metabolites. Patients are usually treated with a
`diet restricted in tyrosine and phenylalanine.
`Although it is not common to use an emergency
`regimen in this disorder, deoompcnsation of
`liver function and deterioration of neurological
`function can follow intercurrent infection. It is
`probably beneficial that an emergency regimen
`is given to reduce these problems. The patients
`may also need appropriate treatment for fiver
`failure.
`
`ORGANIC ACIDAEMIAS
`
`(:1) Propiom}: and methylntalonic actdaemia
`Propionic and melhylmalonic acidaemia are
`caused by inherited enzyme defects in the
`pathways of propionate catabnlism. Propionate
`is formed from several sources including the
`amino acids isoleucine, valine, methionine, and
`threonine, by anaerobic bacterial
`fermenta-
`tion in the gut and catabolism of odd chain fatty
`acids. Although amino acids are widely regarded
`as the rnaior source of propionate, only about
`50% of propionate is derived from breakdown
`of amino acids.“ About 20% is derived from the
`gut“ and the rest
`(30%) probably from the
`catabolism of odd chain fatty acids.’ The toxic
`metabolites and the mechanisms responsible for
`all the clinical problems are not well understood
`
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`lrtterrummt illness in inborn! errors ofiirterarediai-_y meralaalinn
`
`139]
`
`primary systemic carnitine deficiency and may
`be helpful
`in reversing decompensation in
`disorders of meditun chain fatty acids. In theory
`carnitine in long chain fatty acid oxidation
`disorders might be harmful, by promoting the
`uptake of long chain fatty acids into mito-
`chondria. Its use requires more evaluation.
`Blood glucose is often used to monitor patients
`with these disorders, but this may give a false
`sense of security as patients may develop
`marked encephalopathy before blood glucose
`concentrations fall: treatment must be started
`before this.
`
`Conclusions
`The use of a well planned emergency regimen
`will not only help prevent episodes of metabolic
`decornpensation, but will
`reduce hospital
`admissions and improve the parents‘ self confi-
`dence. The policy of encouraging the family to
`bring the unwell child to hospital every time is
`intended to be supportive and may initially be
`necessary, but it may add to the burden of care.
`The correct balance needs to be found between
`overzealous intervention and the risk of compli-
`cations. Furthermore, the needs of each child
`and their family must be iudged carefully and
`
`the instructions adjusted to their own individual
`requirements.
`
`We would like to thank Dr Peter Cla ton and Dr Margaret
`Lawson for their constructive criticism o lb: rnnnuscrlpl.
`
`Note: References about the inborn errors have, in general. (gill!
`er
`.
`firlgjven when: II: staterncnl Is not subatantmcd In Stmvcr
`
`I Seriverck, Beaudct M ., Sly W5, Valle D, eds. The metabolic
`bars": ofinlceritari disease. on Fd. New York: Mcfiravr-Hill,
`I98‘).
`2 Newsholme Ell, Lcoeh AR. Binrlmrrntry for the medical
`:4.-r'mm. Clrit-.l:r:sn:r:jol-m W“
`, I983.
`3 Rennie M], Edwards RHT,
`'
`y D, Matthews DE],
`Ineasuned by stable isotope techniques:
`the e ects of
`'
`Wolman SL. Milward D]. Muscle protein synthesis
`feeding and listing. Cl'urScr' I9!l2;63:S I9—2.l..
`4 Thorn
`GN, Bu IL, Walter ]H, rt of. Protein and
`leucrne nmabolisan in
`syrup urine disease. Am J
`.l"A3asr'ol l99ll:25-l:65-l-60.
`5 Thompson GN, lfrancis DEM, Hnlliday D. Acute illness in
`maple s3rn_rp trnnedrsease: dynamics Df])[D(EIIll roetabollsm
`;I£E4lIl'll|fll.ClIIlII.S for Inanguncnt. _7 Pndrarr
`l99I;ll9:
`6 'l‘hornpsoI1 GN. Walter IH. Bresson IL, er al. Sourors of
`propiooat: production in inborn errors of pmpiomt:
`|rm'aboIisIn.Mcm5ol'r'sru l99£l;!!:]I33—?.
`2-’ Shin D. Prudhom C. ‘Thompson GN, Marinui A, Sauduhray
`JM, Bronson IL. Possible contributions of odd chain fatty
`acid oxidation to propionate pmduction in Inethylmalonic
`and
`ionic acidarrnio. Pediarrfles l992;3l:I88A.
`B D'Angro , Leornrd. JV, Dilun M]. Renal tubular function
`lil'l59_I'I:3lI|yImablll¢ scidaemia Eur J Pecfiarr
`I99I;l5ll:
`
`Appendix Mmrlrfnrrrrrrtd product: used‘ in maple syrup mine disease (MSUD) and mm cycle dimrderr (HUD)
`Product
`rlrrnrw acids
`Energy
`Carfiohydrwte
`For
`Vitammr and Irrirmulr
`Corrurleufl
`trim” 5)
`(Ital!-'00:‘)
`ls!-*9!’ zi
`(gm?!-‘xi
`leurine,
`Ml Hopi: syrup rrulr disease:
`liaise prudwt: coruairr all rulirmr acid: except lulu:
`lrrrrm:.’ied' cba-irr ones,
`fillilllfl MSUD (ll
`l5'5
`462
`54
`23
`Fill rang:
`
`Isolrrmhlta Itlld valinr
`Illfilll funntlla
`
`Maxalnaid MSUD (I)
`Maximum MSUD (I)
`_
`MSUD And (I)
`MSUD I
`(21
`MSUD 2 (2)
`MS'UDdielpou-der{3)
`(B) Urea cycle
`Dialamine (I)
`UCD l (2)
`UCD Z (2)
`
`Full range
`-:05
`51
`300
`30
`Full range
`<:0'5
`34
`290
`47
`_
`.
`.
`_
`l..II'IIl'l.l(‘l rarry
`Nil
`Nil
`520
`%
`Fa! range
`Nil
`29 (sucrose)
`290
`49
`FII range
`Nil
`22 [sucrose]
`Slfl
`65
`Ful range
`2|)
`63
`483
`I2
`I-new produit curtain £l tuixnw: of ersrrrliol and JIM!-¢5Jell«l‘I:tll' amino acids
`30
`am
`62 (54 g sucrose]
`Limited range, low content
`Nil
`Utangrtflavoumed, high sucrose contcnl
`68
`For iruiutts: requires carbohydrate and
`260
`81) (sucrose)
`Nil
`Full range
`for
`to be added
`SI
`For children
`290
`6'0 (sucrose)
`Nil
`Fu! range
`Manufacturer: (1) Scientific Hospital Supplies (SI-IS), Liverpool; (2) Milu
`, Hillin
`, Middlesex: (3) Mend Johnson Nutrilioualvr. I-Iotulslmr, Middlesex. In the UK:
`On]
`the SHS
`erline Sllbsllnoes and can be prescribed on an FPIO.
`oducts have been approved by die Advisory COI‘llI‘I.'I'|l)::¢
`'1
`=II'l8 k .
`
`Suital:|e,:|mr|nfl; gears of age,
`Soluble fidm Illyy-ears of age.
`mod w|9!=i|iIr
`_
`Low solubility [not suitable
`for Ctlnlinutms Feeds)
`For inlnnls: higlt sucrose oontenl,
`requires far to be added
`For I.'l'Lilt:l.ten
`Inlant formula
`
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`
`ADC
`
`lntercurrent illness in inborn errors of
`intermediary metabolism.
`M A Dixon and J V Leonard
`
`Arch Dis Chiid 1992 67: 1337-1391
`doi: 10.1136i’adc.67.11.1387
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