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Penicillin allergy

Allergy

Children with pneumonia with a label of penicillin allergy were found to have:

  • higher risk of hospitalization (RR 1.15)
  • acute respiratory failure (RR 1.27)
  • and need for intensive care (RR 1.46; 95% CI, 1.15-1.84)
  • increased cutaneous drug reactions (RR 2.43)

[US Journal of Allergy & Clinical Immunology in Practice.  11(6):1899-1906.e2, 2023 Jun.]

Cephalosporins

Atanaskovic-Markovic et al found that cross-reactivity between cephalosporins and penicillins varied between 0.3 and 23.9%, being higher among penicillins and between first-generation and second-generation cephalosporins.

However, it has recently been shown that all penicillin allergic children can tolerate cefuroxime, presumably as it has a different side chain.

Cross-reactivity appears to be higher in immediate reactions, and when penicillins and cephalosporins are identical or similar in the R1 side chain, as happens with the first and second-generation cephalosporins.

Currently BNFc says to avoid cefalosporins if history of immediate penicillin hypersensitivity, but if use of cefalosporin is “essential” then can be used (but not cefalexin!).

Canadian study did oral challenges for non-blistering rashes – safe – but mostly cefprozil allergy (and linked to food allergies).

De-labelling

In hypothetical case of de-labelled patient, 47% of anaesthetists would not prescribe penicillin to patient anyway (n=5000)!

Primary care don’t always remove label even after de-labelling! (patient held records would help…)

Needs culture change in primary care and paeds of documenting reactions!

Make sure note is added to patient record when de-labelled. Electronic labels don’t necessarily help – not always possible to remove an allergy label from drug prescription system, depending on the system, may only allow subsequent note to be added.  Free text systems do not encourage accurate description!

Alabama trial from NIHR to report on RCT of de-labelling in primary care; SPACE study in secondary care (nurse/pharmacist delivered).

See Testing for antibiotic allergy.

Lyme Disease

Cardiology, General paediatrics, Infectious disease, Microbiology, Neurology, Rheumatology

Borrelia (spirochaete) infection, spread by ticks (Ixodes), common in localized areas of Europe and North America (forest environments).

Differential includes possible co-infection from other tick born organisms viz anaplasmosis, or babesiosis.

Vaccine available if likely to be at risk.

Clinical

Infection occurs a minimum of 48 hours after bite!

Skin – Erythema migrans is the classic skin lesion, a spreading ring usually at the site of the bite but can be multiple and at different sites.  Typically not hot, itchy or painful. Takes a while for central clearing to develop. Develops over 1-4 weeks (from 3 days to 3 months!), can last months.  Looks like erythema multiforme, but time scale different.  Insect bite hypersensitivity/superinfection looks similar but usually hot, itchy and/or painful, and develops/recedes within 48 hours!

Lyme lymphocytoma is a painless bluish red nodule or plaque, especially on the ear but also reported on the nipple and scrotum.  More common in children.  May persist for months, can precede other features.  Acrodermatitis chronica atrophicans (ACA) is almost exclusively seen in adults, predominantly women, and is an eruption with chronic, progressive red or bluish-red lesions, usually on the extensor surfaces, with later atrophic, fibroid or sclerodermic changes.

Consider Lyme as possible (but unlikely) cause for:

  • fever and sweats
  • swollen glands
  • malaise
  • fatigue
  • neck pain or stiffness
  • migratory joint or muscle aches and pain
  • cognitive impairment, such as memory problems and difficulty concentrating (sometimes described as ‘brain fog’)
  • headache
  • paraesthesia

Arthritis – uncommon, presents as recurrent inflammation of 1 or more large joints, usually the knee. Swelling can be disproportionate to pain.  Can become more persistent – in a minority, despite treatment, inflammation becomes chronic (presumably immune-mediated).

Carditis occurs rarely, and almost always with other clinical features.  Usually partial heart block, but can be complete, usually resolves within a week.

Neurological – isolated facial palsy, meningitis, other cranial nerve palsies, meningoencophalitis, polyradiculopathy.  There is a small proportion of children who can present with non-specific headache, fatigue, neck pain without clear neurological signs (and also the rare case of raised intracranial pressure).

Other rare disease manifestations include uveitis, iridocyclitis and keratitis.

Diagnosis

For erythema migrans, clinical diagnosis is adequate, and antibodies only positive in 30-70% anyway!

Use a combination of clinical presentation and laboratory testing to guide diagnosis and treatment in people without erythema migrans. Do not rule out diagnosis if tests are negative but there is high clinical suspicion of Lyme disease.

  • Offer an enzyme-linked immunosorbent assay (ELISA) test for Lyme disease – consider starting treatment with antibiotics while waiting for the results if there is a high clinical suspicion. (Test for both IgM and IgG antibodies)
  • If the ELISA is positive or equivocal, perform an immunoblot test for Lyme disease (again, consider starting treatment with antibiotics while waiting for the results if there is a high clinical suspicion). [Western blot increases specificity, but cut offs (for both serology and Western blot) can be an issue, with potential false positives for other acute infections and autoimmune conditions.  Definitely needs to be an approved lab…]
  • If ELISA negative and the person still has symptoms, review their history and symptoms, and think about the possibility of an alternative diagnosis.  If tested within 4 weeks from symptom onset, repeat the ELISA 4 to 6 weeks after the first test.
  • If Lyme disease is still suspected in people with a negative ELISA who have had symptoms for 12 weeks or more, perform an immunoblot test.  If negative, consider synovial fluid aspirate/biopsy, or lumbar puncture [PCR – culture is difficult – or CSF antibodies for neuroborreliosis; consider for isolated facial palsy]
  • If immunoblot negative and symptoms resolved, no treatment is required.

For early neuroborreliosis, antibodies 80% sensitive, rises to virtually 100% for late or ACA.

Early antibiotic treatment is also believed to potentially block antibody production.

Antibodies can then persist for months or even years after successful treatment of the infection, so repeat testing is not useful for monitoring treatment success.

First line ELISA test can have false positives for other spirochaetes, glandular fever and autoimmune conditions.

The idea that there are seronegative “chronic Lyme” cases has little evidence to support it, with only 2 possible cases reported (ACA and arthritis, not neuro).

NICE says “Discuss the diagnosis and management of Lyme disease in children and young people under 18 years with a specialist, unless they have a single erythema migrans lesion and no other symptoms. Choose a specialist appropriate for the child or young person’s symptoms dependent on availability, for example, a paediatrician, paediatric infectious disease specialist or a paediatric neurologist.”

Treatment [check NICE]

The most commonly recommended first-line treatments for different stages of Lyme borreliosis in Europe are:

  • Erythema migrans/borrelial lymphocytoma:  10-14 days Doxycycline if 9yr+ (initially 5 mg/kg in 2 divided doses on day 1, then 2.5 mg/kg daily in 1–2 divided doses, max dose 200mg, for a total of 21 days, option for higher dosing) – 10 days courses of doxy effective in US trials.  Else Amoxicillin 50mg/kg/d, max 500mg TDS (10-14 days)[BNFc says 30mg/kg/d, max 1g, TDS for 21 days].  Don’t delay treatment pending test results.  Scandinavia use 10 days Pen V (100mg/kg/d, max 1000mg TDS). BNFc says Azithromycin as alternative.
  • Isolated facial palsy: 14 days Oral doxycycline  – else as above.  Doesn’t probably help resolution but may prevent later complications.
  • Meningitis/radiculopathy: PO Doxycycline or IV Ceftriaxone  50-100mg/kg/d, max 2g daily (14-21 days). [BNFc talks about CNS disease separate from cranial/peripheral nerves]
  • Encephalitis, myelitis: Ceftriaxone (14 days)
  • Lyme arthritis: Doxycycline (28 days) else Amoxicllin (21-28 days)
  • Carditis: Ceftriaxone during pacing, else PO doxycycline (14 days)

Ceftriaxone is the most commonly preferred parenteral agent, with once-daily dosing facilitating outpatient treatment. Recent prospective studies have shown that oral doxycycline is noninferior to ceftriaxone in neuroborreliosis, and it is now recommended in Europe for the treatment of acute facial palsy (FP), meningitis and radiculoneuritis. Ceftriaxone currently remains the preferred choice for children with other presentations of neuroborreliosis and for those with contraindications to doxycycline.

Several recent EM treatment studies have incorporated noninfected control groups. Excellent responses were seen, with resolution of rash within 7–14 days. Nonspecific symptoms including headache, myalgia, arthralgia, fatigue and parasthesias were no more common in cases than controls at 6-month follow up.

[position statement by the British Infection Association, J Inf 2011;62:329]

[Pediatric Infectious Disease Journal Volume 33(4), April 2014, p 407–409]  

Clostridium difficile

Gastro, Infectious disease

Classically causes pseudomembranous colitis.  A soil anaerobe, spore producing.

In children, most will have had antibiotics within past 4-12 weeks, other risk factors are past and/or prolonged hospitalization.  Many comorbid conditions associated esp cancer, inflammatory bowel disease. Clindamycin was classically the antibiotic most associated but any will do.

There may be fever and abdominal pain.  Colitis can mimic (or exacerbate) IBD.  Severe infection unusual.  Markers established in adults for severe infection resulting in colectomy or transfer to ICU have not been shown to correlate in kids.

“Pseudomembranes” are a non-specific endoscopy finding.  Not always tested for automatically by lab, you may need to specify. Toxin test (not 100% sensitive).

Current Opinion in Pediatrics. 26(5):568-72, 2014 Oct. PMID 25032717

Meningococcus W

General paediatrics, Infectious disease

MenW now appears to be endemic in England, with cases now in young children, and across all regions.  Previously used to only be seen in epidemics, particularly related to Haj travel.

Numbers now about 1/4 those of MenB.

Mortality rate approx 12%.  Some adult cases of septicaemia progressing rapidly to death.  Some atypical presentations eg arthritis, severe pneumonia, GI symptoms without non-blanching rash (still rapidly progessive).

JCVI have recommended vaccinating all UK adolescents aged 14-18 years of age (school years S3-S6 in Scotland) with MenACWY as soon as practicable, this is in addition to introduction of Bexsero® vaccine (MenB) for all infants in Scotland.

Ehlers-Danlos syndrome

General paediatrics, Genetics, Orthopaedic, Rheumatology

Covers a spectrum of problems, classified by Beighton (as in hypermobility, Marfans etc). “Vast genetic heterogeneity and phenotypic variability” – 2017 proposed classification includes 13 subtypes!

Classically:

  • joint laxity (hypermobility),
  • skin hyperextensibility,
  • tissue fragility (easy bruising, skin splitting, hernias, prolapses).

Stretchy skin can feel soft and doughy.  Associated with atrophic scars (that look like bad healing).

Mild myopathy seen.

  • Type 1 is severe, commonly born prematurely due to premature rupture of membranes, joint and skin laxity are gross with frequent orthopaedic problems. History of hernia repair? Aortic root dilatation and mitral valve prolapse have been reported but evidence on prognosis is still conflicting.
  • Type 2 is milder and so often underdiagnosed.
  • Type 3 is benign joint hypermobility without skin problems!
  • Type 4 is the rare but severe form where risk of arterial rupture, mostly between ages 20-40. Autosomal dominant so may be history, sometimes characteristic facies. Abnormal bruising is characteristic and the skin is unusually translucent but paradoxically it is not especially hyperextensible, and hypermobility may not be very obvious (perhaps only in fingers)! Problems rarely present before age 20 so important to pick up. Arterial rupture can affect anywhere – aneurysm or bizarre fistula may precede, else trauma or surgery may be a trigger. Bowel rupture is often seen although not usually lethal, pregnancies can be affected by uterine rupture +/- haemorrhage.

Sheffield does genetic testing.

Coeliac disease

Gastro, General paediatrics

Autoimmune process triggered by gluten, distinct from type 1 IgE mediated wheat allergy –  leads to subtotal villous atrophy in terminal ileum. Multifactorial, genetics important – at least 90% positive for DQA1*05 and DQB1*02 alleles that code (confusingly) for DQ2 and/or the DQB1*03:02 allele that codes for DQ8 molecules.  The risk of having coeliac disease without these variants is less than 0.1% so good negative predictive value, but very little positive predictive value – you may not have it now, but you may well get it in the future.

1 in 200, mostly undiagnosed/adult, decreasing in children. More girls than boys, 10% risk to 1st degree relatives.

Randomized trial of early introduction of gluten (4 months, rather than UK recommended 6 months) in 1300 babies found not a single case of coeliac disease at age 3, cf 7 cases in later introduction group (JAMA Ped 2020) – similar result for egg/peanut allergy (same EAT study).

Signs

Become less convincing the older you are! Can present even in infancy, although diagnosis may be delayed for a year or more.

Consider in patients with:

  • Chronic or intermittent diarrhea
  • Chronic constipation not responding to usual treatment
  • Chronic abdominal pain
  • Distended abdomen
  • Recurrent nausea, recurrent vomiting

Should also be considered for these extra-intestinal symptoms:

  • Weight loss, failure-to-thrive, stunted growth/ short stature
  • Delayed puberty, amenorrhea
  • Irritability, chronic fatigue
  • Neuropathy
  • Arthritis/arthralgia
  • Chronic iron-deficiency anaemia
  • Decreased bone mineralization (osteopenia/osteoporosis), repetitive fractures
  • Recurrent aphthous stomatitis
  • Dermatitis herpetiformis–type rash
  • Dental enamel defects
  • Abnormal liver biochemistry

And consider for these specific situations:

  • First-degree relatives with CD
  • Autoimmune conditions: T1DM, thyroid disease, liver disease
  • Down syndrome, Turner syndrome
  • William’s-Beuren syndrome
  • IgA deficiency

[ESPGHAN guidelines 2020]

Diagnosis

Biopsy is no longer essential for diagnosis in children with high serum IgA class antibody concentration against transglutaminase 2 values (10 times the upper limit of normal) with appropriate tests and positive endomysial antibodies (EMA-IgA) in a second serum sample, even in absence of symptoms. Shared decision making with parents recommended.

Children with positive IgA class antibodies but under 10x ULN should have biopsy.

Beware IgA deficiency (associated with coeliac disease, which gives false negatives for IgA TTG – our lab can see this when they do the test, so no need to request total). Also good to check FBC, ferritin, folate, LFTs, Calcium, prothrombin time.

“HLA typing is not required in patients with positive TGA-IgA, if they qualify for CD diagnosis with
biopsies or if they have high serum TGA-IgA (>=10x ULN) and EMA-IgA positivity. If a patient tests negative for HLA DQ2 and DQ8, the risk of CD is very low, while a positive result does not
confirm the diagnosis.”

“Recent studies suggest that the no-biopsy approach to diagnose CD can be applied in asymptomatic children. In asymptomatic children, however, the PPV of high TGA-IgA >=10x ULN may be
lower than in symptomatic children, which needs to be considered during the decision-making process”

  • Positive tTG alone is insufficient for diagnosis, and should not be a reason for gluten free diet.
  • If tTG is high but less than 10x upper limit, then proceed to duodenal biopsy.
  • If tTG more than 10x upper limit of normal, alternative to biopsy is to check IgA EMA and do HLA DQ2/8 screening. If BOTH positive, diagnosis is confirmed. If EMA not available, a second strongly positive tTG is an acceptable alternative (but save serum for EMA testing at future date).
  • If IgA deficient, use IgG tTG or EMA, but less specific so low threshold for biopsy.

Note that antibodies may be false negative if the diet is low in gluten at the time of testing. So normalize gluten intake (at least 2 meals per day, for at least 6 weeks) but discuss with specialist if clinical condition so poor that treatment cannot be safely delayed.

False positives also occur, so diagnosis must be confirmed by endoscopy (or another diagnosis may become apparent). Endoscopy for high risk symptoms (diarrhoea, wt loss, anaemia) and serology is 100% sensitive for coeliac. But a proportion with high risk symptoms and positive serology do NOT have positive biopsies! Cause? Perhaps atypical disease (ie no GI symptoms) is actually more common than typical! Consider the incidences of anaemia, infertility, short stature, unfavourable pregnancy outcomes…

Screening

Coeliac disease is associated with other conditions, which may justify screening:

  • Type 1 diabetes (8%)
  • IgA deficiency (up to 7%)
  • Downs, Williams and Turners syndromes!
  • Autoimmune thyroiditis
  • Autoimmune liver disease
  • Unexplained abnormal LFTs
  • Relatives of coeliac disease patient (10% in 1st degree relative)

Before testing, discuss relative risks of untreated coeliac disease (osteoporosis, infertility, small bower cancer), and the need for biopsy and gluten free diet if tests are positive. Then test:

BSPGHAN say check HLA DQ status and IgA tTG.  NICE says do not use HLA DQ2/8 in non-specialist settings, but consider if not proceeding to biopsy or those on low gluten.

  • If HLA DQ2/8 neg, then v unlikely.
  • If DQ pos but tTG neg, then unlikely (unless IgA deficient or on low gluten diet). Still potential to get disease later in life, so test again in 3yrs or if symptomatic.
  • If DQ/tTG pos but tTG less than 3x upper limit of normal, then do IgA endomysial antibody (EMA) and proceed to biopsy if positive. If EMA negative, continue to monitor.
  • If DQ pos and tTG higher than 3x, biopsy. (Although option is given to retest in 3-6 months)
  • If IgA deficient (<0.07mg/L), consider using IgG EMA, deamidated gliadin peptide (DGP), or tTG
  • Have a low threshold for retesting if signs/symptoms consistent!

Biopsy

Villous atrophy on biopsy (do 4) is characteristic. Marsh grading system: infiltrative changes with crypt hyperplasia is compatible, positive serology strengthens diagnosis. If diagnosis still uncertain, consider HLA testing as above, repeat biopsy after increasing gluten intake or else go the other way, and biopsy after gluten free diet!

Biopsy if gluten excluded and persistent high titres at 12 months or persistent symptoms.  Serological tests alone not adequate to determine if gluten has been excluded!

Treatment

The benefits of treatment, in symptomatic children are:

  • resolution of symptoms,
  • reversal of bone demineralisation,
  • Resolution of micronutrient deficiencies and probably better height gain
  • less delayed puberty, menstrual problems, subfertility, spontaneous abortions and LBW babies
  • decreased risk of some intestinal cancers, to normal levels (T cell lymphoma, Ca oes/pharynx).  T cell lymphoma risk falls to baseline after 10 years gluten free diet
  • Possible prevention of associated autoimmune conditions (evidence conflicting) – see below

In asymptomatic patients, there are no long term studies as yet to show benefits so need to discuss with family.

Diet

Food labelling allows up to 200ppm gluten in gluten-free foods: some people may be sensitive at lower levels than this (at least, their mucosa may be sensitive – they may remain asymptomatic). Gluten free foods are available on prescription.

Oats are safe for 95% of patients (besides contamination issues and the above food labelling problem). Ideally exclude initially and consider reintroduction (products marked gluten free) when well eg at 1yr; normalization of tTG and continued low titres reassuring!  NICE says can be included at ANY stage, but review immunological/clinical/histological response.

Most coeliac patients can tolerate wheat starch and malt extract but not all.

Follow up

If compliant on diet, antibodies should go negative within a year. The mild transaminitis commonly seen at diagnosis should resolve – if not, and on diet, then consider Autoimmune Hepatitis. Complete mucosal recovery can take years.

Monitor symptoms, growth, puberty, antibodies (eg 6 monthly, then 12-24 monthly). Poor clinical response = poor compliance! TTG, FBC, Ca/Phos/ALP, Ferritin, Vit B12, folate, Vit D, PTH, TFTs.

Consider bone scan – see osteoporosis

Discuss oats, as above.

Pneumococcal vaccine recommended.

Transition

Understanding of diagnosis, antibodies.

  • Encourage maturation of communication and decision-making skills.
  • Allow patients to take responsibility for medical self-management.
  • Help the patient develop healthy habits and self-care skills that encourage autonomy eg smoking, exercise, alcohol/drugs
  • Education and counselling re: gluten-free diet (food on prescription, nutritional completeness, healthy weight) and consequences of non-adherence.
  • Recognition and treatment of psychological/emotional issues eg : discouragement, feeling overwhelmed, anxiety about the future and complications such as depression and eating disorders.
  • Familiarize young person with healthcare system

For those patients with significant pubertal delay, paediatric provider may be better suited to provide guidance until transition to an adult provider at the completion of puberty.

Topics for discussion –

  • How there can be a long interval between gluten exposure and the return of symptomatic disease.
  • How coeliac disease can interfere with school, education and work (eg military service)
  • Sexuality and fertility (women with untreated CD are more likely to suffer an adverse pregnancy outcome) – lifetime fertility however is similar in individuals with and without coeliac
  • Adolescents report lower adherence than younger children, esp at social events. Dietary non-adherence is associated with poorer QoL and increased physical symptoms. Most young people with CD think that avoiding cancer is the most important reason to adhere to diet, but the risk for cancer is much lower than previously presumed, so osteoporosis and adverse pregnancy outcome may be bigger issues.
  • Risk of passing it on to children.
  • Associations with diabetes, thyroid disease, autoimmune liver disease.

Primary care may be a suitable care provider.

[Prague consensus, Ludvigsson Gut 2016;65:1242-1251.]

Coeliac UK support group

Mediation

Generic

Has to be voluntary to work! Do both parties want resolution?  Can they express reasons for discomfort/distress?  Can mediator control and sustain process (needs to be safe)?  Are all parties committed to keeping agreement in long term?

“Everybody does that” – groups allow individuals to fail to take responsibility for behaviour.

Ideally fix teams before getting to point of mediating between 2 (or more) individuals!

Issues typically:

  • inequity/fairness
  • annoyance
  • hurt (esp respect, valued)
  • disagreements (even though healthy!)
  • competition

Good attention – focus, openness, impartiality, caring, withholding judgment.

Stages – before meeting face to face.

  1. Tell story, compare before and after, what changed?  But acknowledge qualities in others (start to shift thinking!).  Define issues.
  2. What could a future look like?
  3. How do we get there?  What is most important to you?  What could you live with?  Honesty essential!

Then write introductory statement before sitting down face to face.

(Andrew Graham, Diane Swanson)

Values based reflective practice

Generic

VBRP (Michael patterson)

Reflection vs quality control, reporting back to management!

Bitching=reflection without looking to future!

Should be a process of turning history into learning.  Purpose is to reflect on practice, with a view to fostering habit of reflecting IN practice.

Start is traditionally with Actual Practice.  VBRP starts with Motivations (Soul and role, vision and values.  Why bother!?).  Sorting that out tends to deal with problem of quality control!  From Actual practice, then to Potential practice.

Needs conducive environment cf patronising, judging, analysing.  Else survival but not learning.

2 tools available – 3 levels of seeing, and NAVVY.

3 levels of seeing

•    Observe without interpreting (don’t hold back!)
•    Questioning/curiosity (I wonder…  Do you ever…)
•    What do you think now? (Penny drops – perception, realization)

Offensively simple, but hard to do without diving in.

NAVVY

•    Needs – whose needs were met?  Not met?
•    Abilities – what does situation tell us about abilities/capabilities?
•    Voice and power – Who was heard?  Not heard?
•    Values – what was undervalued?  Overvalued?
•    You – what does situation say about you/me/us?

What’s the matter with you? cf What matters to you?

“Nothing about me, without me” (mental health)

Can be applied to ward rounds, MDT, training/education, service delivery etc etc

Research has shown increased motivation, satisfaction, confidence and reduced stress.  Even better team communication (shared values?).

Enuresis

Common, General paediatrics, OPD
  • Primary or secondary – have they ever been dry? Only considered secondary if consistently dry for at least 6 months
  • Neurology – dribbling, ankle jerks, anal tone
  • Daytime or night time

Nocturnal wetting is considered normal up to the age of 7.  Quantify daytime wetting – pants only, patch on clothes, or puddle.

Diagnoses to consider: (not mutually exclusive)

  • Spinal lesion
  • Excessive urine production – diabetes, lack of mature pattern of ADH production at night
  • Excessive bladder tone/poor bladder volume – urgency, posturing
  • Abnormal voiding – straining, intermittent or poor stream. Could be a bladder neck problem, or else incomplete emptying.
  • Inadequate nocturnal awareness

Diaries of input/output, wetting/waking and measuring overnight urine output may be helpful. The expected bladder capacity is (Age + 1)x 30, max 390ml. If night time urine output is substantially greater than this eg 130% of expected, then suggests nocturnal polyuria. Similarly, day time voiding of consistently less than 65% of expected capacity suggests a small bladder. Going 8x a day or more (with a normal fluid intake) is another clue.

Recommended fluid intake (NICE):

  • 4-8 yrs: 1000–1400 ml
  • 9–13 years: 1200–2100 ml (boys sl more!?)

Incomplete bladder emptying can be defined as post void residual urine volume of greater than 20 ml, on more than one ultrasound, without excessive bladder distension before hand.

For bladder problems, exclude UTI/diabetes by urine dipstick testing.

For bladder or bowel problems, look for any signs of constipation, not just hard/painful stools.

Daytime wetting or urinary urgency/frequency

Ensure adequate fluid intake.  Aim for at least 6-8 cups of appropriately sized water-based drinks spread throughout the day (e.g. 200ml for a 7 year old, about a teacup full;  250ml for an 11 year old, about a mug full).  Start with 8 small drinks every day and increase the amount gradually so the bladder gets used to being stretched.

Try avoiding fizzy drinks, blackcurrant, orange, and drinks containing artificial colourings, flavourings and sweeteners, tea/coffee/hot chocolate for a few weeks, then introduce them one at a time to see what effect they have on the bladder.

Aim for 4-7 voids per day.  If child tends to hang on for prolonged periods, ensure adequate fluid intake as above, then consider an alarm to remind them to go (vibrating watch available, £40+).  Do not encourage excessively frequent voiding – bladder will then become less able to contain normal volume.

Check child is properly relaxed going to the toilet.  Feet should be supported if sitting, use child toilet seat if tending to slip through.  Boys should try sitting for some pees each day, as well as standing.  “Don’t push your pee out.  When you think you’re finished, count to 10 and start again.  Tell your teacher if no toilet paper/soap or broken seat/locks etc.” (Snakes and ladders booklet, Kidney Kids Scotland).

Oxybutinin for small bladder (Desmopressin may work but less rational). Tolerterodine, Solifenacin are alternatives but still antimuscarinic, so same side effects.

Night time wetting

Deal with daytime wetting or urgency/frequency first.

Ensure adequate fluid intake through the day, spread evenly through the morning and afternoon/early evening.  Stop drinking an hour before bed time.

Fully empty bladder before bed time.  Try going twice!

Make toilet easily accessible in night – lower bunk, night light, bucket etc

Do not encourage regular or random lifting/waking – there is no evidence that this promotes long term dryness!  Should only be used as temporary short term measure.  Where night time wetting has not responded to management, a young person may find it useful to set an alarm for themselves.

Establish reward system for drinking well through day and helping to change bedding/pyjamas (rather than for staying dry, which is beyond their control).  Choose appropriate goals, choose appropriate reward (choices/time, not necessarily monetary/dietary), choose appropriate format. Dot to dot picture rather than calendar?

Think positively – say  “I can wake up and go to the toilet if I need to in the night!” before going to sleep. Try without nappies/pull-ups from time to time.

Get bed pads or waterproof mattress protector

Consider bed wetting alarm – bedpad or pant sensor? Buzzer or beeper? Alarms from ERIC cost £50-150!  NICE says avoid alarms if doesn’t suit household, emotional stress esp parental blaming, or if infrequent wetting. Will disrupt sleep, of course!  Parents may need to help child wake to alarm, need to do consistently and chart progress.

Offer trial of desmopressin if child over 7yrs, especially if rapid short-term improvement a priority, or if alarm not suitable.  Consider from age 5yrs.  Response rate low for those without obvious polyuria.

Refer to ERIC website (www.eric.org.uk) or helpline  (0845 370 8008) for further support/information

Although primary nocturnal enuresis is still reasonably common up to the age of 7yrs, addressing the issues above should nonetheless be considered in children younger.

Refer to Secondary Care:

  • Bladder dysfunction: straining to pass urine, intermittent or poor stream
  • Abnormal neurology or lumbosacral spine (naevus, hairy patch, pits, asymmetry)
  • Social/emotional factors that are affecting likelihood of improvement
  • Bedwetting not responding to alarm or trial of desmopressin
  • History of recurrent urinary tract infection
  • Day time wetting not improving with first line advice as above

See ERIC (Childhood continence) website for parent information. Hjalmas, J Urol 2004 International evidence based strategy for nocturnal enuresis. International Children’s Continence Society.  NICE guideline 111 nocturnal enuresis

ECG

Cardiology, General paediatrics

Standard ECG settings are 25mm/s and 10mm/mV.  Beware small complexes, which may indicate someone has adjusted the Y-axis to 5mm/mV.

Axis @ birth 60-180, @ 1 yr 10-100, @ >1yr 30-90 (NB prems have LESS Rt dominance!). So you expect QRS to be positive in both I and aVF. Under 1, I can be negative but aVF should still be positive.

RVH defined as:

  • Dominant RV1 +/- Q after 1 yr,
  • Upright TV1 over 1 week and under 7-8 yr,
  • SV6 over 15mm if under 1yr or over 5mm if over 1 yr.

Brugada syndrome – genetic arrhythmia. Persistent or intermittent right sided ST elevation and RBBB, leading to VF and sudden death.

QTc is normally under 0.490 up to 6 months, or 0.425 if over 6 months. Calculation = QT/SQR(RR), where QT is time from beginning of Q to end of T. Easiest way to calculate it is to count small squares: QTc is then QT/5 divided by SQR(RR) (where default is 25mm/sec). Over 450ms with history of syncope is a red flag; otherwise over 460ms. Consider family history of sudden death or “epilepsy”, too.

PR 80-160ms (so 3-5 small squares), QRS less than 75ms. Look for delta wave if PR short.

RV1 less than 20-26mm (trough at 1 year), SV6 less than 10mm at birth falling to 4 mm at 10 year.

LVH – deep Q waves in V6 are a clue (upper limit is 0.54mV, ie 5 small squares). The SV1+RV6 upper limit ranges from 3.1mV (newborns) through to 5.7 (older children), staying at around 5 for most children (ie 50 small squares, my calculations). Glasgow guideline says if V5 (rather than V1) and V6 complexes overlap but this depends on how the ECG is printed and can very between 20 and 40 squares!!

For adults, there are many different criteria for LVH eg:

  • Sokolow + Lyon (Am Heart J, 1949;37:161)
    • S V1+ R V5 or V6 > 35 mm
  • Cornell criteria (Circulation, 1987;3: 565-72)
    • SV3 + R avl > 28 mm in men
    • SV3 + R avl > 20 mm in women

But sensitivity of ECG criteria less than 20% at specificity levels of 88% to 92%. Obesity affects chest lead voltage, for example.  Better in patients with a specific cardiac disease. An elevated LVM (left ventricular mass) index is taken as the reference for LVH. In kids the SV3R + RV7 Sokolow-Lyon parameter performs best, but who does V7 routinely? (and still only 25.3% sensitivity).

So you can say voltage criteria for LVH met, but can’t say diagnostic of LVH.  More likely when other features such as left axis deviation, ST and T wave changes. ST elevation under 2 squares not significant. T wave inversion in V5/6 most suspicious (Glasgow guideline). When clinical evidence is also taken into account, the sensitivity improves considerably (but still under 50%).

[Normal ranges – European Heart Journal (2001) 22, 702–711]

LVM (as estimated from echocardiographic measurements) is itself vulnerable to measurement error and may oversimplify the geometry of the left ventricle. Alternatively, a combination of increased LVM and clinical evidence of volume or pressure overload of the left ventricle may be a better reference standard.