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Infant Feeding and Nutrition Notes_PClarke_140907

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    Digestion and nutrition.

    Introduction

    History

    Where growth or nutrition problems are suspected, take a careful feeding

    history. Ask the parent to tell you everything the child has eaten and drunk in

    the last 24 hours, talking them through the meal times and asking about

    snacks and drinks between meals. Ask specifically for quantity of milk and

    squash drunk and how many portions of fruit and vegetables are eaten daily.

    Are there battles over the childs eating! "oes the child eat alone or with

    other family members! A food diary kept by the parents over a # or 4 day

    period is sometimes useful.

    Table 1 Infant feeding history

    History CommentsWhich milk $reast or formula. If formula note which one and

    details of reconstitution.%ow much feed. In breast fed infants does mother have a good

    milk supply! In formula fed infants note volumes

    offered and taken.%ow often &ote the times of feeds in the previous 24 hrs

    %ow long does the feed

    take to complete'haracteristics of feeding. %ungry, windy, apathetic, slow, sleepy etc.

    (ey symptoms of gastrointestinal problems in children are vomiting, diarrhoea

    and abdominal pain. )*able 2+

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    Table 2 Symptoms of gastrointestinal problems

    Symptoms Enquire about

    omiting olume - dribble onto clothes or a stomach full

    &ature - effortless, forceful )onto child or parent+,

    proectile

    )several feet away+, single vomit or run of vomits

    /requency

    0elationship to feeds and posture.

    1resence of blood or bile in vomit."iarrhoea %ow often - a bowel action after each feed is common if

    breast fed

    'onsistency - pure liquid, porridgey or mied teture,

    undigested food

    'olour

    1resence of blood or mucus'onstipation %ow often, is wind passed between times

    %ow difficult - straining to go or with3hold, painful

    oiling

    'hilds attitude to the problemAbdominal

    pain

    ite5radiation!

    1attern of onset and relationship to food, stress, medication

    'olicky or constant

    duration

    Waking the child at night )this implies an organic cause+

    6amination

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    tart by looking generally at the child and their nutrition. "o they look thin!

    Are there loose folds of skin in the groin or around the buttocks! &ote the

    health of hair, skin, nails and teeth and look for aundice and pallor

    7ently eamine the abdomen. 6plain what you are going to do and get the

    child to tell you if it hurts. Watch the childs face for signs of discomfort as you

    eamine. "ont forget to eamine hernial orifices and the genitalia in boys with

    acute abdominal pain. *he following scheme is recommended8

    General points

    Assess the childs growth - is there poor weight gain!

    9ook for aundice or anaemia

    Assess the childs muscle bulk - wasting of the gluteal muscles is seen in

    coeliac disease.

    Is there any finger clubbing )see *able #, p:+

    Any evidence of assisted feeding such as a nasogastric tube or a

    gastrostomy

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    Inspetion

    9ook for abdominal distension. If present is it symmetrical!

    Are there any operative scars!

    9ook for hernias

    Inspect the genitalia. "o they look normal. Are there any signs of puberty!

    !alpation

    9ook at the childs face while palpating the abdomen. tart palpating at a point

    furthest away from any tenderness. A reluctant child may be put at ease if their

    own hand is used, with the eaminers hand overlying.

    /eel for any tenderness. Is there any rebound tenderness or guarding!

    Identify any enlargement of the liver, kidneys or spleen. *he liver should be felt for

    along a path from the right iliac fossa to the right hypochondrium. *he spleen

    should be felt for on a path from the right iliac fossa to the left hypochondrium. It is

    common to feel the liver edge and the tip of the spleen in younger children. ;any

    children have a more prominent liver edge with concurrent respiratory disease as

    hyperinflation pushes the liver down.

    *he lower pole of each kidney may be felt by palpating with both hands 3 one

    behind the flank pressing forwards towards the other .

    /eel for other masses. 'ommonly faeces are palpable in the left iliac fossa.

    'onstipation is common, and may be associated with tenderness over the caecum

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    due to gaseous distension. In severe constipation the whole lower abdomen may

    be distended.

    !erussion

    'an be useful in delineating the edge of the liver or spleen and in eamining for

    ascites.

    "usultation

    9isten for bowel sounds in the acutely unwell child.

    0ectal eaminations in childhood

    *his is never a routine eamination. It is rarely helpful. 'areful thought must be

    given to undertaking this eamination in any child.

    "ssessment of Gro#th

    Well infants are often weighed regularly to reassure parents and professionals.

    0esults are recorded in the 1ersonal 'hild %ealth 0ecord - usually a 0ed $ook in

    the ? of birth weight needs assessment. $irth weight usually doubles by 4

    months and trebles by =2 months. 0eview the childs growth in length, head

    growth and weight gain. /ailure to grow in any of the parameters is of concern, but

    weight lossneeds urgent investigation at any age. A child whose weight crosses

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    two centile lines is @failing to thrive and nutritional, physical or psychological

    causes should be sought.

    Infant feeding

    $reast /eeding

    *he is no doubt that breast is bestB and with the correct support, advice and

    encouragement the maority of babies will thrive on breast feeding until at least 4

    months of age. *he milk is easily digested with low antigenicity and anti3infective

    properties. $reast feeding is good for mother child bonding, convenient and free.

    1roblems and 'ontraindications to breast feeding are very few )see table #+

    Table $ !roblems #ith breast feeding

    !roblem %anagement

    /eeding difficulties upport mother )%ealth isitor, ;idwife,

    support groups+Inborn errors of metabolism 0are, require specialist advice;aternal drug5alcohol abuse 'areful monitoring of infant;aternal %I 'ontraindication in developed countries;aternal medication 'heck safety in $&/ or with pharmacist1rematurity 6pressed breast milk given via &7 tube*uberculosis 'ontraindicated

    $reast fed babies feed on demandB. *his will be at least every 23# hours for the

    first few weeks, gradually increasing to every #34 hours by day and :3C hours

    overnight by a few months of age. $abies who are getting enough breast milk

    settle after feeds, have :3C wet nappies per day, tend to pass soft stool quite often,

    may be after every feed, and show growth on their centile charts.

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    $reast milk is nutritionally complete for term infants until 43: months of age.

    ;others who are unable or choose not to breast feed directly should be actively

    encouraged to epress. 6pressing should be started as soon as possible after

    delivery to take advantage of the normal physiological changes that occur after the

    birth.

    $ottle3fed infants should be fed on demand, to start with a term infant will take D>3

    E>mls E or C times per day, increasing to =C>322>mls D times a day by # months of

    age. *eats are available and different types suit different babies.

    Alternative milks

    'ows milk based formulae are modified to resemble breast milk as closely as

    possible. tandard /ormula are 4>? casein :>? whey and those marketed for

    %ungrier InfantB are C>? casein 2>? whey. tandard formulae are suitable up to

    =2 months of age and the casein based feeds have no proven benefit. oy milks

    are not recommended under : months of age due to concern about levels of

    naturally occurring plant oestrogens. /urthermore, 2>? of infants with cows milk

    allergy show cross reactivity with soy protein.

    7oat or sheep milk is not recommended due to high solute load, similar

    immunogenic potential to cows milk and concerns about sterility.

    %ypoallergenic formulae are recommended for infants with milk allergy or

    considered at risk for developing it. *hey are either highly hydrolysed cows milk

    )hydrolysed feeds+ or composed of amino acids )elemental feeds+.

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    Infant weaning

    *he best time for weaning appears to be 43: months of age, even if the baby was

    born prematurely. 0enal and gastrointestinal immaturity means earlier

    weaning is inadvisable, and delaying longer seems to increase the risk of

    feeding difficulties. *he World %ealth Frganisation guidelines suggest

    eclusive breast feeding until : months, which is appropriate to developing

    countries where risk of infection is greater.

    Weaning foods should be introduced gradually, a new one every few days, starting

    with very simple vegetable purees or baby rice. *he compleity and range

    of tastes and tetures are gradually increased. *here should be no added

    salt or sugarG gluten and eggs may be introduced from : months. 'ows

    milk is not used as the main drink until = year of age but it may be

    incorporated into solids from : months. It is recommended that full fat milk

    is given until at least 2 years of age. Alternative formula milks )follow onB

    milks+ are available with higher levels of iron, vitamin " and protein

    content.

    Diet for older hildren

    *he principles of healthy eating are a diet that is roughly 4>? carbohydrate, #D?

    fat and =D? protein, and includes five portions of fruit and vegetables per day,

    plenty of fluids, ideally water rather than squash and other soft drinks, and refined

    carbohydrate in moderation.

    itamins

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    itamin deficiency syndromes are very rare in the developed world ecept for

    children with malabsorption. itamin A deficiency is still the most important cause

    of blindness in the world. itamin A supplementation can also reduce the risk of

    death from infectious disease.

    itamin " deficiency causes 0ickets. In the

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    1arenteral &utrition

    Intravenous feed containing carbohydrate, fat, protein, minerals, vitamins and

    trace elements are used primarily as an adunct to enteral feeds or short term

    replacement for those who cannot tolerate enteral feeds, for eample after bowel

    surgery. /ive to ten per cent of children will have complications from the

    intravenous feeding line, including sepsis, blockage and embolic phenomenon.

    1rogressive impairment of liver function may occur with long3term parenteral

    nutrition, and may culminate in cirrhosis and liver failure.

    The &omiting Infant

    omiting in infancy is a fact of life Almost every baby will posset, bringing up a

    small amount of milk after a feed, often with wind. It is necessary to distinguish this

    from significant gastro3oesophageal reflu or vomiting due to obstruction, or from

    neural causes. *he case scenarios describe some characteristic histories and their

    causes.

    7astro oesophageal reflu

    Anna, a six month old infant presented with a history of vomiting since birth. What

    had initially seemed to be harmless positing of small amounts after feeds has

    progressed to bother the whole family. The vomiting was worse after feeds or if

    she was being moved or carried but could occur at any time. It seems effortless

    with enough produced to stain everyones clothes and the floor in the vicinity. The

    vomit containing partly digested milk. The clinical picture of a fraught family with a

    cheerful wellnourished thriving baby was characteristic!

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    7astro oesophageal reflu is usually a benign disorder which resolves

    spontaneously between si and =2 months of age as infants spend more time

    upright, solids are introduced and natural anti3reflu mechanisms develop.

    0eassurance is the mainstay of treatment. 1arents can try smaller volumes of milk

    given more frequently and positioning the infant with head elevated after feeds.

    /eed thickeners may help.

    9ess commonly, reflu is severe and infants may fail to thrive. Fesophagitis may

    develop. 0eflu is commonly troublesome in infants with cerebral palsy, broncho3

    pulmonary dysplasia and milk intolerance.

    Investigation

    A barium swallow and meal will usually confirm reflu and may identify

    abnormalities of oesophageal motility, strictures or hiatus hernias.

    6ndoscopy will enable confirmation of oesophagitis, and stricture formation.

    It is useful for diagnosis of reflu associated with allergy )eosinophilic

    oesophagitis+ which may not be associated with significant acid reflu.

    A p% study will confirm the severity of reflu by demonstrating the duration

    of acid reflu into the lower oesophagus.

    *reatment options include dietary substitution, antacid therapy, prokinetic drugs or

    surgery )fundoplication+.

    1yloric stenosis

    Adam, a six week old infant presents with a " day history of progressive and

    dramatic vomiting which does not contain bile. This occurs during or after meals

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    and the vomit is pro#ected across the room so carers have learned to keep their

    distance. $espite the vomiting the baby seems hungry, feeding avidly until the

    vomiting occurs. %n examination the baby has lost weight, is constipated and

    mildly dehydrated.

    1yloric stenosis describes a pathological thickening of the pyloric sphincter, which

    hypertrophies over the first few weeks of age. *his causes obstruction of the

    gastric outlet. It affects =8=D> males and =8ED> female infants. It is more common

    in first3born children and in those with an affected parent.

    "iagnosis can be made by a test feedB. *he hypertrophied muscle is palpable

    during a feed. isible gastric peristalsis may be seen through the abdominal wall.

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    *eeding was stopped, a nasogastric tube sited, and intravenous fluids

    commenced. As she had previously passed meconium, an intestinal atresia was

    excluded. 'he had an urgent abdominal +ray which showed a paucity of bowel

    gas, suggesting upper small bowel obstruction. 'he was transferred urgently to a

    paediatric surgery centre, where, after a diagnostic contrast study showed

    malrotation, she underwent urgent surgery with fixation of the caecum to the

    posterior abdominal wall, and appendicectomy.

    $ile stained vomiting is an indicator of serious surgical pathology, and requires

    urgent assessment. Abdominal J3rays may reveal evidence of obstruction with

    fluid levels and dilated small or large bowel, or may show a displaced caecum

    indicating malrotation as in this case.

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    followed later by restoration of bowel continuity, with a pull3throughB procedure,

    typically around a year of age. 'hildren with "owns yndrome are also at greatly

    increased risk of %irschsprungs "isease.

    0arely, if only a very short segment of aganglionic bowel is present, the

    main symptom is constipation )see below+, however in this case, symptoms will be

    present from very early life, usually from the neonatal period.

    Strangulated herniasmay also produce bilious vomiting secondary to intestinal

    obstruction.

    /eeding problems

    A careful history will usually establish the cause and eclude straightforward

    overfeeding. Infants with nasal obstruction, palate problems may feed messily,

    take in ecess air and vomit. Irritable babies may gulp feeds and vomit. ;ilk

    intolerance may cause vomiting, with or without diarrhoea and pain )see below+.

    7astroenteritis

    'rinath, a formula fed child aged months, developed acute diarrhoea and

    vomiting over a few hours. -e vomited all oral fluids and had four bowel actions in

    as many hours. This was associated with fever and listlessness. -is older brother

    had a similar, but less severe attack ( days previously. xamination revealed

    moderate dehydration. The abdomen was soft with active bowel sounds.

    7astroenteritis is a common infectious disease. ;ost cases are viral, and rotavirus

    causes over D>? of cases. $acterial causes include almonella, 'ampylobacter,

    6.coli and higella, and may cause a dysentery3like picture, with blood and pus in

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    the stool, and high fever, commonly with abdominal pain. %owever, abdominal

    pain is not usually a prominent feature in viral gastroenteritis. Infection with 6. coli

    F=DE may lead to a very severe gastroenteritis, which may be complicated by

    acute renal failure.

    Dehydration

    "ehydration results from depletion of electrolyte3rich gastrointestinal secretions

    through vomiting and5or diarrhoea. In response to these losses there is

    redistribution of water by osmosis throughout the intra3 and etracellular fluid. *his

    leads to a reduction in plasma volume and stimulation of thirst. 'onsumption of

    hypotonic fluids depresses etracellular sodium concentrations, reduces plasma

    osmolality and A"% secretion, and reduces thirst, even if alimentary and other

    losses continue. *he renin3angiotensin system is stimulated, conserving sodium,

    and maintaining blood pressure. *hus in mild to moderate dehydration )D?+, there

    is minimal electrolyte disturbance, but the child will have reduced skin turgor and

    lethargy and reduced urine output.

    As losses continue, severe dehydration results, with features of shock, including

    tachycardia and hypotension. *he etremities will feel cold. *he child is obtunded

    through severe dehydration with accompanying hyponatraemia.

    'hildren who cannot drink sufficiently, or who have disproportionate loss of water

    - such as febrile infants, may develop hypernatraemia in response to dehydration.

    %ypernatraemic dehydration is associated with irritability, and in infants a full

    fontanelle, but tissue turgor is maintained )the skin has a doughy consistency+,

    and thus the severity of dehydration may not be appreciated.

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    In formula fed infants a short break from milk feeds may aid recovery - breast fed

    infants should continue to feed normally. If unable to do so, the mother should

    epress milk to avoid impairment of subsequent lactation. ;ild and moderate

    dehydration is best treated with oral rehydration solutions containing glucose and

    sodium chloride, given little and often. In infants and young children, these may

    be administered via nasogastric tube if necessary. evere or hypernatraemic

    dehydration requires prompt correction of shock and administration of

    glucose5saline solutions appropriate to the electrolyte status of the child. Acidosis

    is a common accompaniment of dehydration through a combination of lactic

    acidosis from impaired peripheral perfusion, and ketogenesis from rapid lipolysis.

    *ypically this responds well to simple or intravenous rehydration or although

    correction with sodium bicarbonate may occasionally be required.

    0ecovery within 4C hours is the norm. *he development of a secondary cows milk

    protein intolerance or lactose intolerance may lead to persistent diarrhoea which

    can be managed using a non3cows milk formula, or a lactose free formula, for =32

    months.

    Intussusception.

    /asey, a child of six months presented screaming with bouts of colicky abdominal

    pain and pallor. The pain was so bad that the she seemed transiently distant and

    obtunded in the aftermath. There was some vomiting followed by the passage of

    bloody stool with mucous, likened to redcurrant #elly. A sausage shaped

    abdominal mass was palpable in the abdomen.

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    Intussuseption in which the bowel partly invaginates, leading to ischaemia,

    characteristic redcurrant elly stoolsB and severe episodes of pain with pallor, and

    vomiting, often causes diagnostic confusion. *he highest incidence is seen

    around :3K months of age.

    It may mimic gastroenteritis, and even meningitis because of obtundation. 'hildren

    may be dehydrated or shocked. ometimes an identifiable lead point is found at

    the point of intussusception such as a polyp, ;eckels "iverticulum, or enlarged

    1eyers patch. *his occurs more commonly in older children with intussusception.

    *he infant may appear well between episodes of pain. *he diagnosis may be

    suggested on abdominal J3ray, and has a characteristic ultrasound appearance.

    *he intussusception may usually be reduced by a contrast enema using air or

    liquid contrast medium. *his should be performed in a paediatric surgical centre.

    urgery is indicated for failed reduction )2D?+, perforation, or recurrence )43=>?+.

    ystemic disease

    Almost any systemic disease can present with vomiting including upper respiratory

    tract infection, raised intra cranial pressure, urinary tract infection, metabolic and

    psychological disorders. Always eamine the child carefully, and obtain a clean3

    catch urine sample.

    "bdominal pain

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    Acute appendicitis

    0icky, a seven year old boy had a history of vague central abdominal pain after

    school. -e ate little supper and spent a restless night during which he vomited

    once and passed a small loose stool. 1y the morning he had significant colicky

    central abdominal pain and would not eat. 1y the time he saw a doctor ) hours

    into the illness the pain has begun to localise to the right iliac fossa.

    %n examination he looked unwell with a dry furred tongue, offensive breath and

    slightly sunken eyes. -is pulse was raised at 22 per minute and his temperature

    was 3).4/. -e was holding himself awkwardly bent slightly and to one side and

    complained of pain on movement. -is abdomen was tense and tender all over but

    ex5uisite in the right iliac fossa, with rebound tenderness.

    *his boy had acute appendicitis and surgery was performed as soon as possible.

    *he history above is typical but it can be difficult, especially in the preschool

    children, or if the appendi is unusually sited. Fther causes of acute abdominal

    pain must be considered, see table D.

    Table ( Causes of aute abdominal pain

    Appendicitis;esenteric adenitis*onsillitis5

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    /rystal, a ( week old infant was taken to see her 67 because she had been

    crying inconsolably every evening for a week. 'he was feeding well and thriving

    with no other concerns. -er parents felt there must be something seriously wrong

    although she was well at other times of the day.

    If history and eamination reveal no worrying features it is highly likely this baby

    has colic. 'olic is ecessive crying in a healthy infant which starts within the first

    few weeks of life and lasts until around 43D months. trictly speaking, this means

    at least # hours of crying a day, on # days a week for at least # weeks but it is

    used much more loosely to cover crying that parents perceive to be unacceptable.

    *he cause of colic is not known but painful peristalsis, trapped wind, lactose

    intoleranceG dietary protein intolerance and parental aniety have all been

    suggested. It has also been regarded as a behaviour pattern with no pathology.

    *here is no evidence that herbal remedies, gripe water or simethicone reduce

    colic. In bottle fed babies a =32 week trial of a cows milk protein free formula may

    be of benefit. 0eassurance is vital. Acknowledge how stressful the crying is, and

    that it will improve with age.

    &on specific abdominal pain

    8ibby, a 9 yr old girl attended outpatients with a 9 month history of bouts of colicky

    central abdominal pain. $uring attacks, which were common after school, she

    looked pale and miserable and had to lie down for 32 to :2 minutes. %n other

    days she repeatedly complained of abdominal pain. When 5uestioned she said

    she always had pain. 'he was missing a lot of school. %ver the counter remedies

    were ineffective. -er appetite was normal and there was no weight loss, vomiting

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    or alteration of bowel habit. 'he was growing normally. There were no abnormal

    signs on examination.

    Abdominal pains like this affect =>? of children and can have a variety of labels

    including &A1 )non3specific abdominal pain+, functional abdominal pain and

    sensitive bowels. ;any children will have no worrying features in the history or on

    eamination and do not require investigations. In others inflammatory bowel

    disease, food intolerances or other causes )see table : may need to be ecluded.

    Table ) *ther auses of reurrent abdominal pain.

    Cause +ey linial features0enal or ureteric pain. 1ain eperienced laterally or posteriorly.7astritis or 7F0 6pigastric or chest pain.

    1eptic ulceration. &ight pain.pinal or back problems. 9ateral pain. igns over the spine.

    )*enderness, limited movement+0enal or gall bladder disease. 0ight hypochondrial pain. Laundice.'onstipation.

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    peers. *he stresses in their lives are usually common ones, such as trying to work

    in a noisy classroom and arguments with friends. "ont forget to ask about

    bullying, but dont assume all children with pain are facing some huge problem in

    their lives About half of these abdominal pains settle within a few months. In some

    the pains can be recurrent and a proportion will go on to develop irritable bowel

    syndrome as an adult.

    ;esenteric adenitis

    'entral abdominal pain which is associated with tenderness, occurring at the

    same time as an

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    passed the hard impacted stool, and through a stretched anal sphincter. *his is

    very demoralising to child and families and the risks of social isolation and school

    avoidance are real. 'onstipation is rarely due to a food intolerance, endocrine

    causes, such as hypothyroidism, or neurological causes. In short3segment

    %irschprungs disease, symptoms date back to the neonatal period.

    'onstipation is manageable with enthusiastic and supportive therapy. A

    combination of good diet, stool softeners and osmotic laatives will produce a soft

    stool. timulant laatives and behavioural modification will produce regular

    emptying.

    Chroni Diarrhoea

    'hronic diarrhoea in children has diverse causes ranging from toddler diarrhoea to

    inflammatory bowel disease.

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    Table , Clinial Charateristis of Different Causes of Chroni Diarrhoea

    Abdominal

    1ainomiting

    ;ucous or

    $lood

    7rowth

    /ailure

    *oddler

    "iarrhoea3 3 Fccasional

    mucous3

    /ood

    AllergyM MM M

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    /ood intolerance may be caused by allergy, )cows milk protein+, immune

    mediated disease eg coeliac disease, enHyme deficiency eg lactose intolerance, or

    chemical irritation )chillies+

    Allergy

    'ows milk protein allergy is the most common food allergy, but allergic reaction

    can develop to other antigenic proteins including oya, eggs, peanuts, tree nuts,

    cereals and fish. 0isk of allergic reactions increases if there is a strong family

    history of atopy. ymptoms include urticaria, stridor, anaphylais, vomiting,

    diarrhoea, abdominal pain and behavioural changes.

    Investigations are rarely of value although eosinophilia, raised food3related blood

    Ig6 levels or positive skin prick tests may be found. A therapeutic trial of food

    withdrawal, with symptoms reccurring on subsequent challenge is the gold

    standard for diagnosis.

    ;anagement is to strictly eclude offending antigens from the diet. *wenty

    percent of children with cows milk protein intolerance, also develop symptoms

    with soya, so hypoallergenic formulae are advised. ;ost children will outgrow the

    allergy by 2 3D years, although in very few it is lifelong. If there is a history of

    severe allergic reactions, particularly anaphylais, food challenges should be done

    in hospital.

    Sugar intolerane

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    *hese are due to enHyme defects and fermentation of undigested sugars causes

    eplosive frothy stools with flatus, with or without abdominal pain and distension.

    9actase deficiency is the most common and is usually transient )after severe

    gastroenteritis or untreated coeliac disease+ or due to a progressive fall in lactose

    production )common in non3'aucasians, who have increasing intolerance with

    age+. ery rarely, it may be congenital, when it is inherited as an autosomal

    recessive trait.

    Coelia Disease

    'oeliac disease is a small bowel enteropathy due to intolerance to the protein

    gluten in wheat, barley, rye and possibly oats. It can occur at any time after the

    introduction of gluten into the diet. *here is a very strong genetic susceptibility.

    *he disease affects between = in #>> and = in =>>> people. *he risk is higher

    with a family history, autoimmune disorders, diabetes and "own syndrome.

    ;ost infants and children with coeliac disease have diarrhoea, which is

    typically pale, fatty, offensive and porridge3likeB in nature. Fther symptoms are

    described in *able E.

    Table Symptoms of oelia disease

    Type of presentation Signs and Symptoms

    'lassical presentation )K3=C months+

    1ale, irritable, failure to thrive,

    Abdominal distension, buttock

    wasting6arly presentation )OK months+ omiting or constipation9ate presentation )childhood3adult

    life+

    Iron deficiency anaemia, short

    stature, fatigue, abdominal pain,

    creening )In patients with insulin

    dependent diabetes or a strong family

    ubtle symptoms or none at all

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    history+

    'oeliac disease is a permanent condition and should not be confused with

    transient wheat or gluten intolerance, which may occur in children under 2y,

    especially after gastroenteritis.

    'linical history and positive blood antibody testing )anti3gliadin, anti3endomysial,

    tissue tranglutaminase antibodies+ may suggest coeliac disease but a small bowel

    biopsy is essential to confirm the diagnosis. *ypical histology shows subtotal

    villous atrophy with increased lymphocytes in the lamina propria and epithelium.

    *he management of coeliac disease is with a strict gluten free diet ecluding all

    wheat, rye and barley. Fats may be tolerated later but are usually ecluded to

    begin with. taple foods can be prescribed by 71s. upport of a paediatric

    dietitian is essential, and the 'oeliac ociety is an invaluable source of up3to3date

    information on gluten3free products. &on3compliance with diet risks osteoporosis,

    infertility and small bowel lymphoma.

    Crohn's disease

    'rohns "isease affects = in =>>> adults, = in 4 of whom present in adolescence.

    It causes a inflammation of the whole bowel wall )transmural involvement+G

    anywhere from mouth to anus, with the terminal ileum being most often affected.

    *he presentation is often delayed with non specific symptoms such as growth

    failure, delayed puberty, anoreia, poor concentration, lethargy, malaise and

    deteriorating school or sport performance. If colitis is present, diarrhoea and

    abdominal pain may be a feature. 6amination may show evidence of malnutrition,

    linear mouth ulcers and perianal skin tags. *enderness or abdominal masses are

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    variable. Inflammatory markers such as 60 usually parallel disease activity, but

    the nature and etent of inflammation need to be demonstrated. *his is usually by

    upper gastrointestinal endoscopy and colonoscopy with biopsy.

    ;anagement begins with induction of remission using nutritional therapy

    )elemental or hydrolysed feed and food eclusion+ or systemic steroids. Fn3going

    maintenance treatment with D Amino3alicylate derivatives )alaHopyrine,

    mesalaHine+, immunosupression )aHathiaprine, methotreate+ or immune therapy

    )monoclonal anti3*&/ alpha+ may be needed. urgery should be avoided

    whenever possible but may be needed for complications such as obstruction,

    fistulas or failed medical treatment. *ypically the course is relapsing into and

    throughout adult life.

    /lerati0e olitis

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    be needed for complications such as toic megacolon, hemorrhage or failed

    medical treatment. urveillance colonoscopy is performed annually when disease

    has been active for => or more years to reduce the very real risk of carcinoma.

    aundie in Children

    Laundice arises from elevation of bilirubin. %aemolytic disorders cause aundice,

    or it may arise from impaired clearance of bilirubin in hepatocellular dysfunction,

    as occurs with hepatitis, or from biliary obstruction, such as biliary atresia.

    The Infant #ith *bstruti0e aundie

    7eter was referred by his 67 at 4" days of age, after his health visitor noticed that

    he was still #aundiced. -is weight gain was poor, and he had dark urine with

    puttycoloured stools. l, the ma#ority of which was

    con#ugated. -e had abnormal coagulation. 1iliary atresia was suspected and he

    was urgently referred to a tertiary specialist centre. 1iliary atresia was

    subse5uently confirmed, and he underwent surgery.

    *he young infant with obstructive aundice requires urgent evaluation to eclude

    biliary atresia. In biliary atresia, there is progressive obliteration of the etra3 and

    intra3hepatic bile ducts, leading to hepatocellular inury, with cirrhosis and portal

    hypertension. urgery to construct a bile conduit )(asai procedure+ is performed

    as soon as possible. If surgery is unsuccessful, then liver failure results, requiring

    liver transplantation. If bile drainage is achieved, then the progression of liver

    disease may be slowed, although ascending cholangitis, malnutrition due to fat

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    malabsorption, and portal hypertension leading to oesophageal varices and

    consequent gastro3intestinal haemorrhage are common complications.

    *he differential diagnosis of obstructive aundice includes congenital holedohal

    yst affecting the etrahepatic biliary tree, and neonatal hepatitis. &eonatal

    hepatitis presents with prolonged obstructive aundice and evidence of

    hepatocellular dysfunction. It is usually idiopathic, but infective, endocrine and

    metabolic causes - notably galactosaemia and alpha3=3antitrypsin deficiency must

    be ecluded.

    A baby with prolonged jaundice

    1ridget was a term baby who had difficulty establishing feeds. 'he presented with

    sepsis at : days of age. 'he had vomiting, diarrhoea, and hypoglycaemia, and

    was lethargic and #aundiced. xamination revealed hepatomegaly. The liver

    en?ymes were elevated, with an elevated con#ugated bilirubin, and abnormal

    coagulation studies. 1lood cultures were positive for 6. coli. 0educing substances

    were present in the urine and 1ridget was found to have reduced levels of

    galactosephosphate uridyl transferase @6A87

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    are common, notably verbal dyspraia. 7irls are almost always infertile due to

    premature ovarian failure.

    Hepatitis

    %epatitis is characterised by aundice with dark urine containing bilirubin and

    urobilinogen, sometimes with other features of hepatocellular dysfunction.

    Hepatitis "is a common infectious disease. *he virus spreads by the faeco3 oral

    route and cause infectious aundice. *he maimum period of infectivity occurs in

    the two weeks prior to onset of aundice. *here is little systemic upset but often

    abdominal pain, vomiting and loss of appetite which may last several weeks.

    *ravellers to endemic areas, or eposed contacts, such as other family members

    at risk of infection, may receive hepatitis A immunisation.

    Hepatitis or Cmay be acquired from contaminated body fluids, or more often,

    from congenital infection. 'hronic hepatitis $ and ' may cause cirrhosis and

    hepatocellular carcinoma. creening of children at riskB of hepatitis $ is

    recommended in the

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    "utoimmune hepatitis and slerosing holangitis are often associated with

    inflammatory bowel disease. *he liver problems may be the first manifestation of

    this. Autoimmune hepatitis is characterised by elevated autoantibodies,

    particularly anti3nuclear, and liver3kidney3microsomal antibodies. 'irrhosis is often

    present at diagnosis, and indicates a poorer prognosis. *reatment is with

    aggressive immunosuppressive therapy.

    *bstruti0e aundie in Childhood

    Fbstructive aundice in older children is seen with choledochal cyst )see above+,

    and gall stones secondary to chronic haemolysis or hypercholesterolemia.

    'hildren requiring long3term parenteral nutrition are at high3risk of progressive liver

    disease and resultant liver failure.

    -ulminant Hepati -ailure

    /ulminant %epatic /ailure )/%/+ is fortunately rare, and manifests with

    encephalopathy, hepatocellular dysfunction )impaired coagulation,

    hypoalbuminaemia with oedema, acidosis, hypoglycaemia+ renal failure and

    sepsis. *he commonest cause in the