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Glomerulonephritis

General paediatrics, Renal

Inflammation in the concentrating tissue of the kidney can produce a range of manifestations:

  • Proteinuria
  • Haematuria (microscopic, ie on dipstick/microscopy only, or frank macroscopic)
  • Nephrotic syndrome
  • Acute nephritis

Protein in urine is not usually symptomatic in itself, the urine may seem more frothy.  It would not be until the loss of a substantial amount of protein leads to hypoalbuminaemia and oedema (nephrotic syndrome) that it would be apparent.

Investigations

Accompanying symptoms will tend to guide you to a diagnosis eg purpuric rash (HSP), photodermatitis and/or arhtritis (SLE), heavy proteinuria without haematuria (minimal change glomerulonephropathy).

Dipstick testing is sensitive for proteinuria and haematuria, but urine protein/albumin:creatinine ratio is more reliable.  On microscopy, red and white cell casts are pathognomic.  Presence of red cells useful to exclude other causes of apparent haematuria.

Complement – some characteristic patterns.

If isolated low C3, and fits with PSAGN, diagnosis is clear.  But check it normalizes in 3/12, else biopsy.

Biopsy – for definitive diagnosis.  Although some conditions have patchy changes so sampling error possible.

Causes

Henoch Schonlein Purpura

Common, Dermatology, General paediatrics, Immunology, OPD, Renal, Rheumatology

or HSP. Usually preschool but any age! Boys more than girls. Vasculitis, leucocytoclastic with IgA predominance. EULAR criteria 2010.

Features:

  • Urticarial rash becoming purpuric but still raised (pathognomic), predominantly on lower limbs, especially extensor surfaces incl buttocks, but sometimes trunk, rarely face (infant).  Accompanying soft tissue swelling, as seen in photo in feet.
  • Arthralgia (not migratory, cf acute rheumatic fever)
  • Abdo pain, diffuse, colicky, often severe – possible intussusception, melaena
  • Proteinuria (30+ mmol/mg albumin:creatinine ratio), 2+ red cells on dip or 5+ on microscopy (or casts) indicating glomerulonephritis, usually asymptomatic cf streptococcal, but a few develop diffuse proliferative lesions with irreversible renal damage.
  • Scrotal rash/bruising common, rarely torsion
  • Rarely encephalitis, seizures, pulmonary haemorrhage

Pathology

Leukocytoclastic vasculitis with glomerular mesangial IgA deposits.  Biopsy of patients with IgA nephropathy identical, and indeed both groups have defective IgA1 glycosylation, which may explain why they may aggregate and precipitate IgA in small vessel walls as well as in the glomerular mesangium.

Complications

Complications and relapse associated with age esp over 6yr. 50% relapse, usually within 6 weeks but can be up to a year later; 50% of those will relapse more than once. Treat with NSAIDs (unless renal involvement!) for joint pain, analgesics for abdo pain. Small study from Turkey found that Ranitidine reduced symptoms.

Steroids (prednisolone 1-2 mg/kg/day for 1-2 weeks) are suggested (grade 2 recommendation) within 3 days of onset of severe abdominal pain (defined as pain requiring hospital admission) or acute GI bleeding – after exclusion of intussusception, of course.

Steroids are also suggested for orchitis, after excluding torsion.

Reviewing 101 children with abdo involvement those who did not receive steroids had an average of 5 days of abdominal pain, whereas all those treated recovered within 24 to 48 hours of starting steroids (letter, J Pediatr Gastroenterol Nutr. 31(3):323-4, 2000).

Abdominal pain is a predictor of renal involvement, so maybe that’s the best reason for giving steroids… (Kidney Int 1998; 53:1755-9).

Renal disease

20 to 90% risk of renal disease, but generally mild (mostly just mild proteinuria and/or haematuria)! Normal urinalysis at day 7 has 97% negative predictive value for chronic renal disease [PLoS One 2012;7:e29512].

About 56% of those children with renal disease develop signs and symptoms of renal disease a week or more after presentation, although generally in first month.  Can be up to 6 months later.  Incidence of renal failure in HSP nephritis is just 2 to 5%.  Risk factors are severe abdominal pain (OR=2.1), age >8yrs (OR=2.7), and relapsed HSP (OR=3).[Arch Dis Child. 2010 Nov;95(11):877-82. doi: 10.1136/adc.2009.182394]

A normal urinalysisNB Children who appear to recover may have significant renal disease many years later. (Lancet. 339:280282, 1992).

If more than 50% crescents on biopsy, then poor prognosis.  Only 1% get that far.

Archimedes in 2012 found 3 RCTs of steroids, treatment courses 2-4 weeks, seemed to shorten duration of abdominal pain, with most obvious effect when used early.

Dudley trial of early steroids (day 7) failed to find any benefit at 12 months (n=352).  Quite a high proportion of drop outs unfortunately, but prob not enough to influence results.  Doesn’t answer question of what to do if severe disease at onset eg nephrotic range.  [Arch Dis Child. 2013 Oct;98(10):756-63.]

Non-randomized prospective clinical trial of 223 kids showed that steroids were effective in reducing the severity of abdominal and joint pain and in treating renal disease – steroids did not prevent the development of nephritis [Archives of disease in childhood. 2010;95:877–82, doi: 10.1136/adc.2009.182394 ].  Previous systematic review suggested that steroid treatment at diagnosis did not reduce the median time to resolution of abdominal pain but did significantly reduce the mean resolution time, and increased the odds of resolution within 24 hours.

There is no proven benefit of corticosteroids in the treatment of established HSP nephritis (2009 Zaffanello systematic review, evidence is poor), but used anyway.

The major risks of corticosteroid treatment in children with HSP are masking an acute abdomen or intussuception, and GI bleeding.

Follow up

UK Kidney association guideline 2022 – for uncomplicated presentation, do frequent dipstick checks, for example weekly for first 4-6 weeks then monthly. BP check at presentation and then if evidence of nephritis. No need for parents to check at home otherwise. 6 months minimum (audit criteria, rather than recommendation).

If hypertensive or urinalysis pos, then do proper urine protein:creatinine ratio, U&Es, MC&S. Isolated haematuria is benign. As soon as urinalysis becomes normal, child can have routine follow up.

Scottish guideline was that persisting proteinuria of + or more needs more frequent follow up eg 2 weekly, 2 monthly then 3 monthly with the second line investigations.

Refer urgently for confirmed hypertension, or nephrotic range proteinuria (P:CR over 200mg/mmol).

Refer for biopsy if persisting severe proteinuria (UP: UC >250 mg/mmol for up to 4
weeks), persisting moderate proteinuria (UP: UC 100–250 mg/mmol for 3 months),
AKI stage 1 or greater (creatinine >1.5 × previous baseline or >1.5 × upper limit of normal for age). Treatment depends on histology and severity of clinical features.

ACE inhibitor is suggested (grade 2 recommendation) for persisting mild/moderate proteinuria.

A systematic review found no risk of long-term renal impairment in children with Henoch-Schonlein purpura with normal or minimal urinary findings without nephritic or nephrotic syndrome or renal failure (Arch Dis Child 2005;90:916-20). If urine analysis is normal at presentation, then test for 6 months after the last symptoms. If there is renal disease at presentation, then the risk for progression seems to be more associated with rising proteinuria during follow-up rather than presentation features. (Am J Kidney Dis 2006;47:993-1003)

Lead toxicity

General paediatrics

No safe blood level has been identified and all sources of lead exposure for children should be controlled or eliminated.

Experts now use a reference level of 5 micrograms per deciliter to identify children with blood lead levels that are much higher than most children’s levels. This new level is based on the U.S. population of children ages 1-5 years who are in the highest 2.5% of children when tested for lead in their blood (National Health and Nutrition Examination Survey (NHANES))

CDC is no longer using the term “level of concern” and is instead using the reference value to identify children who have been exposed to lead and who require case management.

In the past, blood lead level tests below 10 micrograms per deciliter of lead in blood may, or may not, have been reported to parents. The new lower value means that more children will likely be identified as having lead exposure allowing parents, doctors, public health officials, and communities to take action earlier to reduce the child’s future exposure to lead.

What has not changed is the recommendation for when medical treatment is advised for children with high blood lead exposure levels. The new recommendation does not change the guidance that chelation therapy be considered when a child has a blood lead test result greater than or equal to 45 micrograms per deciliter.

Vitamin D

General paediatrics, Nutrition, OPD, Orthopaedic

=colecalciferol.  Essential for bone health.

Obtained from sun exposure to the skin.  Only a few dietary sources – oily fish, cheese, egg yolk, fortified cereals. Diet more important for calcium, of course. Once you apply sun screen, you don’t make vitamin D any more so there is a conflict with the potential for skin solar damage including cancer.

Children under 5 considered a high risk group, along with pregnant, pigmented skin, northern latitudes, wearing concealing clothing, being housebound etc.

2021 Scottish government advice is that everyone consider taking a Vitamin D supplement, particularly between October and March, but that all year round supplementation should be taken by:

  • all pregnant and breastfeeding women
  • all infants and children under 5 years old
  • people who have low or no exposure to the sun, for example those who cover their skin for cultural reasons, are housebound, confined indoors for long periods or live in an institution
  • people from minority ethnic groups with dark skin such as those of African, African-Caribbean and south Asian origin, who require more sun exposure to make as much vitamin D

2017 chief medical officer (CMO) advice – all babies from birth up to one-year-of-age should be given a daily supplement of 8.5 to 10μg vitamin D with Healthy Start vitamin drops being the recommended choice of vitamin, other than babies who are formula fed getting at least 500ml per day, as infant formula already has added vitamin D.

Nonetheless, breastfeeding is preferred – supplementation needed for breastfed infants given lack of sunlight in the UK (probably only useful sun exposure in Scotland between April and September, and between 11 and 3pm).

Children aged 1-4 years old should be given a daily supplement containing 10mcg of vitamin D.

“Healthy Start” vitamins preferred – made for  NHS,  available free to those on income support,  contains recommended dose (approx 300 units) .

Standard prevention dose is 300-400u (10mcg) (neonates), between 400u and 1000u (over 1/12) per day.  Over the counter multivitamins often contain surprisingly little Vitamin D.  Drops, tablets, sprays all available.

Many vitamin D preparations around this dose contain calcium, which may improve efficacy in fracture prevention, but some people won’t like.  Fultium D3 capsules have 800u (20μg) vit D and no calcium.

Symptoms

Aches and pains, delayed walking, seizures and tetany, genu valgum/varum, muscle weakness (incl cardiomyopathy).  The classic features of rickets are bowed legs, rachitic rosary (expanded costochondral junctions), pectus carinatum, curvature of the spine, expansion of the metaphyses at the wrist/ankle, poor dentition.

Testing

Historically deficiency defined as 25hydroxyVitaminD below 25nmol/L. But debated what is optimal.  NICE CKS uses this figure, and defines ‘insufficiency’ as between 25 and 50 nmol/L.

Treatment

Vitamin D3 preferred – D2 sometimes used.

If rapid correction needed eg deficiency with symptoms, fixed loading dose used for 8-12 weeks:

  • 1-5 months: 3000 IU
  • 6 months -11 years: 6000 IU
  • 12yr+: 10 000IU. Single/divided oral dose totalling 300 000IU can be considered if compliance issues.
  • After that, standard prevention dose as above unless significant lifestyle changes to improve Vitamin D status.

Otherwise 400-600IU daily from age 1 month to 18 years. Buy over the counter unless for chronic condition that leads to deficiency.

Assess need for calcium supplementation eg milk allergy. Online calcium calculators available.

Colecaciferol liquid available (3000u/ml), tablets come in 1000u doses and higher.  The combined VitD/Cal tablets tend to have lower Vit D doses and may not be tolerated.

Weekly doses, or single megadoses (30x daily dose) have been recommended where compliance a concern. Intramuscular ergocalciferol 7.5mg (300,000 units, 1ml) can be given in special situations.

Alfacalcidol is used in chronic kidney disease, needs specialist advice and careful monitoring.

Monitoring

Repeat measurement of serum 25 OH vitamin D is not usually necessary, and certainly not within 3 months of starting treatment unless agreed with the duty biochemist.  Check compliance eg empty bottles?

Continue supplementation until child has stopped growing.

Vitamin D deficiency in children | Health topics A to Z | CKS | NICE

Bell’s palsy

General paediatrics, Neurology

bells palsyDiverse causes, some of which are extremely serious (mostly infiltrative):

  • Zoster sine herpete ie zoster reactivation without vesicles – probably responsible for a third of otherwise unexplained facial palsies
  • EBV- the most commonly found cause (20%)
  • Ramsay Hunt – look out for soft palate, tongue as well as external auditory meatus lesions
  • Mycoplasma
  • Cat scratch disease, HIV, Lyme disease (erythema chronicum migrans, travel history)
  • Acute otitis media (historically the most common cause!  Reactivation of virus?  Toxin induced demyelination?  But beware mastoiditis!)
  • Trauma
  • Malignancy – leukaemia, cerebellar astrocytoma, rhabdomyosarcoma
  • Histiocytosis
  • Haemophilia
  • Hypertension!

Bell’s Palsy is what you would call it if idiopathic, but perhaps you just haven’t looked hard enough? Often otalgia, facial or retroauricular pain. Typically mild except in Zoster, where it is severe. Pain may precede palsy.

Warning signs (for a serious underlying cause) are:

  • otitis media (in case direct inflammation, ie osteomyelitis),
  • hearing loss,
  • lymphadenopathy, tonsillar enlargement (parotid tumour),
  • mastoid enlargement,
  • frontal sparing, motor function of tongue/fingers (adjacent cortical representation) (UMN lesion)
  • or duration longer than 1 month.

Assess using House-Brackmann scale:

House Brackman criteria

Management

Do hearing test, blood pressure, check frontal sparing/tongue/finger function. FBC if any suspicion of leukaemia. Check blink reflex (prognostic).

Treatment

Apply lubricating drops hourly during day, and an ointment overnight. Patching seems to be frowned upon now.

Treat underlying cause!

For Bell’s, still controversial.  Cochrane review (2016) found that steroids reduce chance of permanent facial weakness (NNT=10), involuntary movements (motor synkinesis) and crocodile tears.  Adding antivirals may improve rate of recovery but low quality evidence – certainly less good than steroids, in fact not much different from placebo when used alone! Even without treatment most make a full recovery within 9 months. (Dundee study in adults, NEJM 2007; 357:1598 PMID 17942873)(Adour, Ann Otol Rhino 1996)(Hato Otol Neurotol 2003, PMID 14600480)

Only 2 studies in children from what I can see.

IV aciclovir in Ramsay Hunt since poor prognosis. Valciclovir would in theory be better, 96% recovery in Hato study (5 day course with steroids cf steroids alone) but unblinded (Otol Neurotol 2007;28:408-13)

Some evidence for methylcobalamin and hyperbaric oxygen.

Recovery usually within 3 weeks, else nerve regeneration takes 4-6 months. Beyond 6 months improvement is unlikely. For long term palsy, feedback training, surgical techniques may result in functional as well as cosmetic improvements. Synkinesis and facial spasm are complications, treat with botulinum. (BMJ 329:553)

Antibiotic classes

Microbiology

Protein synthesis inhibitors

Act on ribosome.  In theory, reduce toxin production as well as growth.

  • Macrolides
  • Aminoglycosides
  • Clindamycin
  • Chloramphenicol
  • Tetracyclines
  • Linezolid

Aminoglycosides

Eg Gentamicin.  Broad spectrum but poor CSF penetration (but still used for Listeria meningitis!).

Some important bacteria are usually resistant to the aminoglycosides, including gentamicin:

  • most streptococcal species (including Streptococcus pneumoniae and the Group D streptococci),
  • most enterococcal species (including Enterococcus faecalis, E. faecium,  and E. durans),
  • anaerobic organisms, such as Bacteroides species and Clostridium species.
  • Salmonella and Shigella
  • Pseudomonas unless you use ones with antipseudomonal activity eg tobramicin

Hearing damage is an important side effect, known genetic marker for this but usually not done ahead of treatment.

Renal excretion.

Quinolones

Block DNA synthesis.  Broad spectrum but not great gram positive, excellent absorption, penetration and intracellular too.  See quinolones.

Vasculitis

General paediatrics, Immunology

Inflammation of blood vessels, clinical manifestation depends on which part of the body (often widespread) and what size vessel affected.

Classic presentations are:

  • rash esp purpuric, pernio (swelling of subcutaneous tissue),
  • ischaemia eg infarction (brain, gut, digits),
  • nephritis,
  • asthma.

The Chapel Hill system provides definitions for 10 different forms of adult vasculitis but is of debatable utility.  EULAR consensus criteria look pretty good [Annals of the Rheumatic Diseases. 65(7):936-41, 2006. PMID 16322081].

Differential: hyperlipidaemia esp severe familial, coagulopathy, fibromuscular dysplasia (familial).

Primary vasculitis

Classified by size of vessel affected (although a degree of overlap often exists):

  • Large vessel ie aorta- Takayasu. May be preceding inflammatory-type illness. ?headache/dizziness, ?inflamm eg Raynauds, episcleritis etc.  Claudication, absent pulses, subclavian bruit.
  • Medium vessel – Kawasaki disease, Wegener’s (C-ANCA pos, nasal/oral lesions, pulmonary lesions, GN, granulomata), Polyarteritis Nodosa (PAN: livedo, neuropathy, aneurysms), Churg Strauss (ANCA pos, asthma, eosiniophilia, neuropathy)
  • Small vessel vasculitis – Henoch Schonlein Purpura (HSP) is the classic one, but also cryoglobulinaemia (?hyperviscosity, assoc with malignancy and chronic viral infection)

Secondary vasculitis

eg SLE, mixed CT, infective esp streptococcus.

Investigations

Characterized by one or more anti-neutrophil cytoplasm auto-antibodies.  These are screened for by immunofluorescence (IF) that can reveal specific patterns of staining, viz pANCA (peripheral staining), cANCA (cytosol staining).  cANCA is predominantly due to Peroxidase 3 antibodies, and is seen in Granulomatosis with polyangiitis (previously Wegener’s granulomatosis, so should be Wanca?!), pANCA is mainly due to Myeloperoxidase antibodies and bacterial permeability increasing factor (BPI), and is seen in 50% of microscopic polyangiitis.

These patterns and antibodies are also associated with rheumatoid arthritis, CF, IBD, drug induced vasculitis, Churg Strauss syndrome, autoimmune liver disease etc.

But in children these are not very sensitive and in any case no evidence that distinction influences treatment or prognosis. The type of ANCA seems to be related to the population rather than the disease! Probably has direct toxic effect.

Imaging is the second modality of investigation, esp MR angiography.

Untreated, these diseases have 90% 2 yr mortality…

Diabetes

Endocrinology, General paediatrics, OPD

Updated NICE guidance 2016.  Characteristics:

  • random glucose >11,
  • polyuria, polydipsia,
  • excessive tiredness,
  • weight loss.

WHO 1999 criteria – fasting normally <5.6, >7 diagnostic.  Random >11 diagnostic (assuming there’s nothing sticky on the finger tested!!!).  Glucose tolerance test (starts with fasting level) 2hr level >11.1 diagnostic, 7.8-11.1 is impaired glucose tolerance.

False positives – infection, recent surgery, uncontrolled thyroid disorder, starvation.

Epidemiology

Increasing rates in Europe. Scotland rate second only to Scandinavia.  Marked increase in under 5s. Aiming for routine pump therapy within a few months.

Subtypes

Type 2 more often in S Asian, Hispanic, Afro-Caribbean.  Clue is raised c-peptide – as this is co-produced with insulin, it means there is still endogenous insulin being produced (and cleared at more consistent rate than insulin, so more reliable, esp if on exogenous insulin).  C-peptide also used to look for insulinoma or factitious hypoglycaemia.  May also predict glycaemia control, complications and response to hypoglycaemic agents.

LADY – latent autoimmune diabetes of the young. More common? Different HLA type. Antibody positive but insulin sensitive and slow progress.

MODY – inability to produce insulin but normal beta cells.  Eg KIR 6.2 mutations, within months of birth.

Management

Reduced ideal HbA1c target – 48mmol/l (6.5%).  

No DAPHNE for kids.

Multiple daily injections from diagnosis, with level 3 carbohydrate (CHO) counting education, blood ketone testing strips.  Other regimens eg BD, TDS only for where problems with compliance.  See also pumps.

NICE guidance now includes Type 2 – suspect if strong FH, obesity, black/Asian origin, minimal insulin requirements (<0.5u/kg after “partial remission phase”), evidence of insulin resistance (viz acanthosis nigricans).

BMJ NICE diabetes infographic

Optimal blood glucose range is 4-7 on waking and between meals; 5-9 after meals; 5+ when driving.  At least 5 tests per day recommended, more frequently during physical activity and illness.But take into account:

  • risk of hypoglycaemia;
  • competitive sports;
  • need to lose weight;
  • life goals (careers, exams, foreign travel);
  • any relevant co-morbidity.

Monitoring

Annual thyroid, hypertension, albuminuria checks from diagnosis; retinopathy testing from age 12.  Type 2 don’t need thyroid but do need dyslipidaemia.

School

Most schools happy to give insulin. But no legal requirement. Lancets for school retract into cartridge.

Self-Efficacy

Encourage ownership of meters etc, downloading at home. Over 14 to get access to SCI-DC, as adults.

Cerebral oedema

Clinical, Emergency medicine, General paediatrics, Neurology

Cytotoxic vs vasogenic (resistant to steroids) vs interstitial (obstruction eg meningitis – not steroids, ?osmotic) vs osmotic (CSF, ECF low osmo) vs hypertensive.

In DKA, there is a 25% mortality from cerebral oedema, 34% long term neurodisability. 

Presents with headache, irritability, agitation (which can be difficult when child is unwell with something else).  Then altered consciousness, posturing, focal neurology (check eye movements, pupils).  Classically Cushing’s triad: hypertension, bradycardia, irregular breathing pattern.

Clinical diagnosis really.  CT can show (better than MRI!).

Hypertonic saline (2.7 or 3%, 2.5-5ml/kg over 10-15 mins) or mannitol (20%, 0.5-1g/kg over 15 mins), some people prefer hypertonic saline but whatever is closest to hand!  Frusemide adjunctive.   

Consider Aciclovir if diagnosis unclear (in case herpes encephalitis – CT not great, LP can be non specific).

Brain protection = 30deg head up, midline position. Avoid hypotension. Avoid hypocapnia (intubate and ventilate if in doubt).

Hyponatraemia common – typically due to SIADH but treat any underlying cause, esp hypovolaemia.

Constipation

Common, Gastro, General paediatrics, OPD, Psychology

Consider constipation if:

  • episodes of faecal incontinence (stains or smears in pants, potentially larger accidents),
  • retentive posturing (standing or sitting with their legs straight and stiff or crossed legs, some will sit on their own heel),
  • occasional massive but soft stools that virtually obstruct toilet.

Not just hard painful infrequent stools! Beware also dyschezia, where baby appears to strain at stool but not actually hard/large.

In a population-based prospective birth cohort, where dietary types were extracted from questionnaire, adherence to a ‘Western-like’ dietary pattern was associated with a higher prevalence of constipation up to 48 months [aOR 1.39; 1.02-1.87], which was not mediated by overweight or sedentary behaviour. Adherence to a ‘Health Conscious’ dietary pattern was only associated at short term, with a lower prevalence of constipation at 24 months (aOR; 0.65; 0.44-0.96). This suggests that specific dietary patterns in early childhood could be associated with higher or lower risks for constipation, but these effects are time-dependent. [Maternal & Child Nutrition. 9(4):511-23, 2013 PMID: 22288911]

Straining is not a criteria, in NICE, interestingly, although it is in Rome III criteria!

Red flags:

  • multiple anal fissures,
  • gross abdo distension,
  • tenderness with guarding,
  • abnormal lumbosacral or lower limb findings,
  • failure to thrive
  • ribbon like stools (?anal stenosis)
  • etc

“Do not use dietary interventions alone as first-line treatment for idiopathic constipation” – because no evidence that it helps! But yes, adequate hydration and fibre important (whole grains, fruit/veg, pulses).

NICE recommends Movicol/Laxido (macrogol) as first line, combining with a stimulant (picosulfate, biscodyl, senna) as second line. If macrogol not tolerated, use stimulant +/- softener (lactulose, docusate).

Warn that pain and soiling gets worse before getting better!

Review of adherence and dose important.

Toilet training eg diary, reward system, regular post prandial sitting 5 mins +/- feet on hard surface eg stool.

“Poo should be as soft as toothpaste and should come out like a snake” (Snakes and ladders booklet, Kidney Kids Scotland). Tell your teacher if no toilet paper/soap or broken seat/locks etc.

Soiling

Typically, episodes of soiling (large and small) are due to overflow of liquid stool past a large impacted stool in the rectum.  The child is unable to control, due to the distortion of the rectum.

However, children often try to deny being aware of soiling, despite the obvious smell or discomfort – this is simply a coping method, and normal sensation is usually easy to demonstrate.

The diagnosis is easier in the presence of a large suprapubic mass, or a rectal mass on digital examination.  Some children however soil for attention, without any bowel or rectal disorder.

The presence or implication of a large rectal mass requires disimpaction – an escalating regimen of a paediatric formulation of macrogol (Laxido or Cosmocol are cheaper than Movicol) as per NICE guideline 99 (doses and licensing may differ from product literature):

  • Child under 1 year: ½-1 sachet daily (non-BNFC recommended dose)
  • Child 1-5 years: 2 sachets on 1st day, then 4 sachets daily for 2 days, then 6 sachets daily for 2 days, then 8 sachets daily (non-BNFC recommended dose)
  • Child 5-12 years: 4 sachets on 1st day, then increased in steps of 2 sachets daily to maximum of 12 sachets daily (non-BNFC recommended dose)
  • If macrogol not tolerated, use stimulant laxative eg picosulfate +/- lactulose

If no progress after 2 weeks add stimulant laxative eg senna, picosulfate, bisacodyl, docusate.

Enemas eg citrate can prevent megarectum where prolonged medical treatment fails.

Polyethylene glycol licensed for distal intestinal obstruction!?

Maintenance

I suggest half disimpaction dose for maintenance.

Preferred treatment is paediatric formulation of macrogol (Laxido or Cosmocol are cheaper than Movicol) as per NICE guideline 99 (doses and licensing may differ from product literature).

  • Child under 1 year: ½–1 sachet daily (non-BNFC recommended dose)
  • Child 1–6 years: 1 sachet daily; adjust dose to produce regular soft stools (maximum 4 sachets daily) (for children under 2, non-BNFC recommended dose)
  • Child 6–12 years: 2 sachets daily; adjust dose to produce regular soft stools (maximum 4 sachets daily)
  • If macrogol not tolerated, use a stimulant laxative eg sodium picosulfate (5mg/5ml, NICE recommended doses):
    • Child 1 month to 4 years: 2.5–10 mg once a day
    • Child/young person 4–18 years: 2.5–20 mg once a day
    • Add lactulose or docusate if stools hard

For babies, Senna and lactulose from age 1/12.

At least 3/12 of maintenance before weaning if disimpaction required initially.

I always highlight that laxative use does not induce dependency, rather, that chronic constipation is unlikely to improve without adequate treatment.

Review regularly – symptoms, toileting, taking medication.

Continue maintenance treatment until regular bowel habit established for at least a few weeks or until toilet trained. Do not stop dose abruptly.

General advice re balanced diet including fruit, vegetables, high-fibre bread/breakfast cereals, baked beans, regular toileting, exercise, sufficient fluid intake (1000-1400ml age 4-8yrs, 1200–2100ml age 9–13yrs).

Involve Health Visitor in pre-school group.

Consider trial of milk exclusion (to rule out cow’s milk protein allergy if intractable (ESPGHAN 2023).   Coeliac disease, hypothyroidism, cystic fibrosis, Hirschsprung’s disease and hypercalcaemia also come into the differential.

Surgery

Rectal biopsy indicated if delayed meconium at birth (ie >48hrs), Downs, enterocolitic episodes.

Anal fissures have high spontaneous healing rate with medical treatment.

Manual evacuation under GA may be required if resistant. No benefit on RCT for anal dilatation. Small RCT found botox as good as internal sphincter myectomy for refractory constipation.

Appendicostomy or caecostomy antegrade colonic enema (where bowel irrigated using catheter) has a role in refractory cases after age 6yrs. QOL, continence improve but appreciable morbidity.

Relapses more common in boys, under age 4, background of psychosocial or behavioural probs, encopresis. 1/3 of post pubertal children continue to have severe problems.  See also parenting and constipation.

Parent information

ERIC website – www.eric.org.uk

[NICE clinical guideline 99 – constipation in children and young people (Published 2010)]

[BMJ 2012]