Paeds Mx 2 Flashcards
Bronchiolitis
- Bronchiolitis is a condition characterised by acute bronchiolar inflammation. Respiratory syncytial virus (RSV) is the pathogen in 75-80% of cases (adeno/myco)
- It is the most common cause of a serious lower respiratory tract infection in < 1yr olds (90% are 1-9 months, with a peak incidence of 3-6 months).
- Coryza for 3 days -> SOB, DRY cough, WHEEZE
Ix: Pulse Ox is first line. Consider ELISA or rapid-antigen testing (RAP) can identify the causative agent which may be useful in infection control of a cohort in a hospital setting.
Immediate referral to hospital:
- Grunting or severe resp distress, marked chest recession, RR>70
- <92% o2 sats
- Central cyanosis
- Apnoea
- Child looks seriously unwell
Consider hospital referral:
- Signs of dehydration (or 50-75% of normal fluid intake)
- > 60 RR
In hospital:
- Largely supportive (oxygen box if <92% sats), CPAP if impending resp failure
- Consider airway suctioning if secretions disturbing feeding or causing resp distress
- Give fluids by NG if they can’t take by mouth.
Cx: Rarely, this causes permanent damage -> bronchiolitis obliterans
Anaphylactic Management
ABC
Airways -> check for obstruction
Breathing -> If not breathing commence CPR + notify advance life support.
If they are breathing, do not commence CPR. Check for circulatory collapse signs and angioedema/urticaria.
Place in comfortable position and give IM adrenaline( 1:1000 into anterio-lateral aspect of thigh. Assess response after 5 min. Repeat in 5 min until there is adequate response.
Give high flow oxygen, fluids (20ml/kg crystalloids)
Do not give IV adrenaline if in primary care. IV chlorphenamine 10mg and IV hydrocortisone 200mg.
- = <6 years - 150micrograms (0.15ml)
6-12 years 300 micrograms (0.3ml)
>12 years 500mcg (0.5ml)
CHOAF - chlorpheniramine, hydrocortisone, oxygen, adrenaline and fluids
Cyanosis immediate mx
• In neonates it is commonly due congenital heart disease
o ABCDE approach
§ Oxygen saturation should be maintained >90%
§ Ventilation e.g. nasal CPAP should be considered
§ Fluids if hypotensive and shock
§ Antibiotics if evidence of sepsis or pneumonia
o Start prostaglandin infusion (5 ng/kg per min) to maintain ductus arteriosus patent.
o If suspected CHD, refer to tertiary care centre immediately
§ Treatment balloon atrial septostomy.
Neonatal Resuscitation Guidelines
Dry the baby, remove any wet towels and covers and start the clock or note the time
• Within 30s : assess tone, breathing and heart rate
• Within 60s: if gasping or not breathing – open the airway and give 5 inflation breaths
o Consider SpO2 and ECG monitoring
• Re-assess: if no increase in heart rate, look for chest movement
• If chest NOT moving: recheck head position, consider 2-person airway control and other
airway manoeuvres, repeat inflation breaths and look for a response
• If NO increase in heart rate: look for chest movement
• When chest is moving: if heart rate is not detectable or slow (< 60/min) ventilate for 30
seconds
• Reassess heart rate: if still < 60 bpm, start chest compressions with ventilation breaths (3:1)
• Reassess heart rate every 30 seconds: if heart rate is not detectable or slow (< 60/min)
consider venous access and drugs (e.g. atropine)
Paediatric BLS
Unresponsive -> Shout for help, open airway, if not breathing normally give 5 rescue breaths.
Check for signs of life for 10s. (brachial and radial pulse)
Still no sign of life, start chest compressions 15 then 2 and repeat. (Speed should be 100-120 compression pm rate)
High risk
Behaviour:
No response to social cues, Appears ill, Does not wake, or if roused does not stay awake, Weak, high-pitched and continuous cry
Tachycardia (different at different ages)
< 60 bpm at any age
Respiratory Rate
§ Tachypnoea (different at different ages), Grunting, Apnoea, SpO2 < 90% on air
Skin: o Mottled or ashen appearance o Cyanosis of the skin, lips or tongue o Non-blanching rash o Aged < 3 months with temperature > 38 degrees o Temperature < 36 degrees
Indications and CI for LP
Contraindications for LP: signs of raised ICP, focal neurological signs, shock, purpura
o Perform LP in the following children with suspected sepsis:
§ < 1 month
§ 1-3 months who appear unwell
§ 1-3 months with WCC < 5 or > 15 x 109/L
Basic prinicipals of shock management
Fluid resuscitation 0.9% saline (20ml/kg) or blood if been in an accident. Repeat if there is no improvement. If still no improvement -> PICU and consider:
Tracheal intubation
Invasive monitoring of BP (arterial catheter)
Ionotropic support (increase strength of contractions)
Renal/liver failure support
Hypoglycaemia management
Initially glucose 10–20 g (o) If necessary this may be repeated after 10–15 minutes. After initial treatment, a snack providing sustained availability of carbohydrate can prevent blood-glucose concentration from falling again.
Hypoglycaemia which causes unconsciousness or seizures is an emergency.
Give IV 10% glucose (maximum dose of 500 mg/kg of bodyweight (5 ml/kg))
If NOT in hospital: IM glucagon or concentrated oral glucose solution (e.g. glucogel). IM glucagon: 500 µg for < 8 years; 1 mg for > 8 years. Seek medical help if blood glucose remains low after 10 mins. Once symptoms improve, give oral complex long-acting carbohydrate.
Raised ICP mx
Head positioned midline Head end of bed titled by 20-30 degrees Intubation/ventilation Mannitol or 3% saline as osmotic diuretics Maintain normothermia and High BP Extreme: shunt
Status Epilepticus Definition
A seizure lasting 5* minutes or longer or when successive seizures occur so frequently that the patient does not recover consciousness between them. *new definition
Status Epilepticus Mx
ABC and DEFG (if glucose under <3mmo/L give glucose IV and recheck blood glucose).
If we don’t vascular access, give a diazepam (PR) or midazolam (buccal) 0.5mg/kg as a trial and aim to get IV access and follow below protocol (only give lorazepam once)l. If you can’t obtain IV access, skip to the PR step.
If you have vascular access, you give lorazepam 0.1mg/kg IV. Repeat in 10 min if no response. If there is still no response in 10 in we give Paraldehyde 0.4ml/kg PR. If there is still no response in 10 min you call for senior help and give phenytoin 18mg/kg IV/IO over 20 min (unless the pt is on oral phenytoin in which case we give phenobarbital 15mg/kg).Call anaesthetist if there is no response in 20min and transfer to PICU for rapid sequence induction with thipoental.
ALTE
Ix and Mx
Essentially a combination of frightening signs that an neonate might exhibit e.g. choking, colour change, apnoea, alteration in muscle tone etc. It might be due to the presentation of a serious disorder or resolve spontaneously. In most it is brief but you need to carry out thorough investigations and monitor overnight. Parents should be taught resus and will find it helpful to have a follow up from specialist peadiatric nurse.
Ix: Thorough screening in case. ECG -qtc conduction pathway abnormality. EEG. Lactate. U&Es. LP. Ba Swallow.
Lactate.
Aetiology: Infections, Seizures, GOR, Airway obstruction
Uncommonly:
Cardiac arrhythmia, Breath holding, Anaemia, Heavy wrapping, Central hypoventilation syndrome, Cyanotic spells
SUDI
Sudden unexpected death in infancy. In some cases, a previously undiagnosed congenital abnormality e.g. congenital heart disease will be found at autopsy. Rarely a inherited metabolic disease is identified e.g. MCAD. After 1 month of age, in most instances, SUDI is attributed and classifid SIDS.
SIDS
Sudden, unexpected death of a young child with no cause found at post mortem. Incidence has dropped dramatically during the last 20 years due to back to sleep campaign where infants are placed on thier back (not side) to sleep, overheating by heavy wrapping is avoided, and they’re placed a the botto mof their cot (so that they dont wriggle down under the blanket and overheat).
SIDS risk factor
Infant Age 1-6 months, peak at 3 months LBW & preterm Multiple births Boy
Parents: Maternal smoking (doubles risk, if a pack a day risk ↑5x) Low income Overcrowded housing Maternal age <20 Single mother, high maternal parity
SIDS Mx
Initiate resus unless inappropriate
Pronounce baby dead
Remove endotracheal tube and IO needles but retain venous line for blood culture and toxicology, metabolic screen ±chromosome screen
Urine culture
LP
Nasopharyngeal aspirate
Break news to parents and explain involvement of police and coroner. Allow them to hold the baby.
Police visit home within 24 hours
Postmortem
MDT where SUDI paedaitrician, police, GP ±social worker review informtion and consider neglecct or abuse
F/U and bereavement conselling
Laryngomalacia
Presents with stridor (exacerbated when cries), afebrile and good weight gain!
Softening (malacia) of larynx, larynx is soft and floppy. Epiglottis is omega-shaped due to weak laryngeal muscle tone. This results in an inspiratory stridor.
This corrects 12-18 months
Ix: Laryngoscopy or Bronchoscopy (looking for omega shape)
Mx: Treatment is rarely required. Surgery sometimes needed where the shortened aryepiglottic folds are cut to correct the omega shape of the epiglottis.
Cx: If a child catches an infection, they can have exacerbation of the stridor and present with respiratory distress (intercurrent infection)
Which of these are linked to Fragile X? MV Prolapse Bronchiectasis Supravalvular Aortic Stenosis Type II Diabetes Mellitus Pigmented Gallstones
Fragile X syndrome is an X-linked dominant trinucleotide repeat disorder. It is the most common X-linked cause of learning difficulties. It can lead to a range of complications including: mitral valve prolapse, pes planus, autism, memory problems and speech disorders.
(bronchiec- Kartagener’s, supravalvular aortic stenosis is Williams Syndrome)
Pertussis Pathogenesis
Bordella Pertussis, g-ve coccobacilli.
Releases 3 toxins that helps adhere the bacterium to the epithelium: Filamentous haemaglutinin, pertactin, agglutin. They also paralyse cilia so that they can’t be swept away, using tracheal cytotoxin. The build up of mucous triggers violent coughing fits.
Pertussin Toxin is also released and this helps adhesion to epithelium and causes a T-cell lymphocytosis via trapping them in blood vessels and preventing them from entering infected tissue. This causes the airways to swell up and makes it harder for pt to breathe causing ‘whooping’ sound.
Infants with pertussis
in infants, cough can be absent but there is apnoea, gasping, cyanosis. Severe hypoxaemia can result in seizures and encelaphathy and secondary pneumonia
Pertussis Management
- Notify HPU
- Azithromycin within 21d of cough onset.
- Clari if neonate
- Admit if under 6 months
- Erythro if pregnant
- Proph abx to household also
- Return to school 48 hours after abx finish or 21d after symptom onset
NICE: In neonates, infants, and children, prescribe 10 mg/kg (to a maximum of 500 mg) once a day for 3 days.
Admission is not needed unless acutely unwell or neonate
Pneumonia organisms
- Newborn – organisms from the mother’s genital tract, particularly group B streptococcus, but also Gram-negative enterococci
- Infants and young children – respiratory viruses, particularly RSV, are most common, but bacterial infections include Streptococcus pneumoniae or Haemophilus influenzae. Bordetella pertussis and Chlamydia trachomatis can also cause pneumonia at this age. An infrequent but serious cause is Staphylococcus aureus
- Children over 5 years – Streptococcus pneumoniae, Chlamydia pneumoniae and Mycoplasma pneumoniae,are the main causes.
- At all ages Mycobacterium tuberculosis should be considered.
Pneumonia Mx + Admission criteria
In CAP, all children tend to be treated with oral amoxicillin 7-14 days as viral can’t be differentiated from bacterial. Children <2 with mild LRTI who’ve had prevenar vaccination do not usually get pneumonia and shouldn’t be given abx, review if sx persist.
Macrolide can be added if sx persist.
Co-amoxiclav is given if pneumonia associated with influenzae.
Only give IV abx if can’t tolerate fluids. (BTS guidelines 2011)
What actually is viral induced wheeze?
- Wheeze is VERY common in pre-school children.
- Has been classified into viral induced wheeze (wheeze when you have the viral URTI, wheeze-free between episodes) and multiple trigger wheeze (as well as viral wheeze they have other triggers)
- Viral induced wheeze is not associated with increased risk of asthma, but multiple trigger wheeze is.
- VIW Mx: Episodic only. SABA -> LKRTA and/or ICS ->
- MT Mx: 4-8 week trial of ICS or LKRTA.
10 puffs every 4hrs.
Asthma Attack Severity
Moderate - Normal Speech 50-70% PEFR
Severe - 33-50% PEFR. Can’t complete sentence in one breath or feed. Accessory muscle use.
Life threatening - <33% PEFR. <92% PaO2. Reduced consc. Exhausiton. Arrthymia. HypOtension. Silent chest.
Mx Asthma Attack
Admit if severe/life threatening
- Give Oxygen to any hypoxia pt and aim for 94-98% sats
1. SABA Is first line
Nebulised with oxygen, 2.5mg if 2-5 or 5mg >5 (use MDI if nebuliser not available)
2. Tiotropium Bromide (10mg <2, 20mg if 2-5, 30-40mg >5)
Other: IV Mg sulphate, IV SABA or IV aminophylline but monitor ECG when adminstering.
Should be given to all children with asthma exacerbation
• Oral prednisolone should be given for 3-5 days
• 2-5 years: 20 mg OD
• 5+ years: 30-40 mg OD
Foreign body inhalation
C: Back blows (x5), Adomdinal thrust (x5) - not to be done in infants
S: Rigid bronchoscopy (stridor or radio-opaque object seen on CXR) or flexible bronchoscopy done under GA
Persistent bacterial bronchitis
o Main organisms: Haemophilus influenzae, Moraxella catarrhalis
o High-dose antibiotics (e.g. co-amoxiclav)
o Physiotherapy
Bronchiectasis
Ix: CT scan (best for bronchiectasis), bronchoscopy may be used if a foreign body is suspected
Mx: Exercise and improved nutrition, Airway clearance therapy (postural drainage, percussion, vibration, use of oscillatory devices)
• Inhaled bronchodilator (e.g. salbutamol)
• Inhaled hyperosmolar agent (e.g. hypertonic saline)
• Long-term oral macrolide (azithromycin)
• Lung transplantation or resection of the bronchiectatic areas
Different presentations of CF
- Newborn - meconium ileus due to thick stool (surgical emergenc)
- Early childhood - Pancreatic insufficiency due to blocked pancreatic duct, causing malnutrition, failure to thrive, steatorrhea, acute pancreatitis and chronic pancreatitis. T2DM secondary to pancreatic failure also occurs.
- Late childhood - Muco-ciliary action is impaired allowing chronic colonisation of bacteria in lungs- ‘CF exacerbation’- and requires Abx. Commonly: Pseudomonas Aeruginosa, Staph Aureus, Aspergillus and Burkholderia Cepacia.
Chronic infection can cause dilatations in bronchial wall (bronchiectasis)
Irritation through blood vessel causing haemoptysis
Ultimately, respiratory failure
4.Other: Infertility in men, nasal polyps, ABPA, digital clubbing.
False positives for CF Ix
Sweat test False positive: malnutrition adrenal insufficiency glycogen storage diseases nephrogenic diabetes insipidus hypothyroidism, hypoparathyroidism G6PD ectodermal dysplasia
too much cl- in sweat due to lack of CFTR so cl- can’t be reabsorbed), false negatives: oedema. Gives an obstructive picture on pulmonary lung function tests.
CF Mx
Mx: Extra calories, replacement of pancreatic enzymes, chest physio to loosen mucous mechanically, inhaler, N-acetylcysteine (cleaves bonds in mucous proteins), Dornase alfa (cuts up nucleic acids in mucous to thin it out) Lumacaftor and Ivacaftor (originally designed for different mutation but are also used to treat delta 508 mutation, luma increases number of CFTR proteins to the csf and Iva potentiates the CFTRs that are already at the surface)
Fanconi Syndrome
Definition: It is a renal tubular disorder characterised by loss of the PCT’s ability to reabsorb metabolites back into the tubular fluid e.g. Go BUPA -> Glucose, Bicarb, Uric acid, Phosphate & Amino acid.
PC: Polyuria, polydipsia, dehydration, Rickets (low phosphate), failure to thrive, failure to reabsorb (alkaline) bicarb results in renal tubular acidosis etc. (hyperchloremic metabolic acidosis).
Other specific transport defects:
Pseudohypoparathyroidism -↑P reabsorp, obesity, depressed nasal bridge, short fingers (2nd, 4th & 5th)
Presenting with Renal calculi (Cystinuria, Hyperuricosuria, Hypercalcuria)
AKI definition
Sudden reduction in renal function classed as oliguria (< 0.5ml/kg per hour)
Nephrotic Definition
> 3.5mg/24hr (proteinuria)
oedema
hypoalbuminaemia
AKI Mx
Use fluid balance charts to monitor intake
If there is circulatory overload, restrict fluids and challenge with diuretic.
Metabolic abnormality management:
HyperK-> SABA, Ca gluconate if ECG changes, Glucose and insulin, dietry restriction
HyperP -> Calcium carbonate, dietary restriction
Met acidosis -> Sodium bicarbonate
HUS -> Anti-hypertensives, 50% of kids will need dialysis
Dialysis criteria: FISH. Failure (of conservative mx or multisystem failure); Increased bp or pul oedema; Severe (acidosis, hypo/hypernatraemia), Hyperkalaemia
Bullous Impetigo
Hydrogen peroxide 1% cream for those who are systemically well
2nd line Abx Fusidic acid (if resistance suspected use mupirocin 2%)
If widespread offer oral fluclox
Eczema treatment steps
1. Creams Emollients all the day Mild - Hydrocortisone Moderate - Betamethasone Valerate (0.025%) Severe - Betamethasone valerate (0.1%)
2nd Line
Topical Tacrolimus
- Bandages fo lichenified skin
- Antihistamines 1 month trial (if severe itching, non-sedative e.g. fexofenadine)
- Fluclox if infected
- o Acliclovir if herpeticum suspected + immediate referral
pediculosis
Itching of scalp or neck. Suboccipital lymphadenopathy.
Identify live lice (on scalp) or empty eggs (whitish oval capsules on hair)
T: direct head to head contact
Cx: There may be secondary bacterial infection over nape (can lead to misdiagnosis of impetigo)
Mx: Wet combing with a fine-tooth comb to remove live lice every 3-4 days for 2 weeks is useful and safe
Dimeticone 4% lotion or aqueous solution of malathion 0.5% is rubbed into the hair and scalp and left on overnight and the hair is shampooed the following morning
Treatment should be repeated a week later
School exclusion is not advised
Scabies
Severe itching, worse at night, Hx of family members itching. Presence of burrows, papules and vesicles on soles and palms (babies) or in flexures of fingers, toes, axilla, wrist and belt line (older children/adults). Incubation period is 2-6 weeks. T: Skin to skin and fomites.
Cx: Itching leads to exorcised skin which can cause secondary bacterial infection or urticarial reaction- can mask + delay diagnosis.
Mx: topical insecticide (permethrin 5% cream)
The product should be applied to the whole body from the chin and ears downwards, esp finger flexures and under the nails
Apply to cool, dry skin and allowed to dry before the pt dresses
Permethrin should be washed off after 8-12 hours (malathion should be washed off after 24 hours)
A second application is required, 1 week after the first application
2nd line: malathion aqueous 0.5% (if permethrin is contraindicated/not tolerated)
Treat entire household
Follow Up:
If persists 2-4 weeks after the last treatment, advice to retreat
Treat post-scabietic itch with crotamiton 10% cream (or topical hydrocortisone)
Night-time sedative antihistamine (e.g. chlorphenamine) may help reduce itching and improve sleep
Viral warts
Rf: occupations which handle fish, meat, poultry
Mx: Spontaneously resolve in most children within 2 years.
Topical Salicylic acid can be used or cryotherapy if refractory.
Firm, rough nodule which is often keratinised. They disrupt natural skin lines.
Ring worm
Tinea capitis (scalp ringworm) causes scaling and patchy alopecia with broken hairs. Examination under filtered UV (Wood’s) light may show bright greenish/yellow fluorescence of the infected hairs with some fungal species.
Rapid diagnosis can be made by microscopic examination of skin scrapings for fungal hyphae. Definitive diagnosis by culture.
Mx:
Keep dry, topical antifungals if mild e.g. clotrimazole, with hydrocortisone 1% cream if needed. If moderate, systemic antifungals e.g. o terbinafine.
Tinea Capitis- Systemic antifungal therapy (e.g. griseofulvin or terbinafine)
Pets who are infected need treatment too.
Psoriasis Mx and Complications
Guttate: Reassure that it is usually a self-limiting condition that typically resolves within 3–4 months of onset, and reassure that it is not infectious. If concerned of appearance: emollient and if v severe a potent topical corticosteroid plus a topical vitamin D preparation (both applied once a day, but at different times of day). Urgent referral to dermatologist for phototherapy if more than 10% of body SA is covered. (NICE)
Guttate: Coal tar preparations are useful for plaque psoriasis in > 6 yrs. Dithranol preparations are very effective in resistant plaque psoriasis. Calcipotriol, a vitamin D analogue, is useful for plaque psoriasis in > 6 yrs
Cx: Occasionally, children with chronic psoriasis will develop psoriatic arthritis
Viral induced wheeze
1st line SABA
2nd line oral montelukast (LTRA) or inhaled corticosteroids or both
Advise parents to stop smoking
Hypotonia causes
An acutely ill child (e.g. septicaemic) may be hypotonic on examination. Hypotonia associated with encephalopathy in the newborn period is most likely caused by hypoxic ischaemic encephalopathy
Central causes Down's syndrome Prader-Willi syndrome hypothyroidism cerebral palsy (hypotonia may precede the development of spasticity)
Neurological and muscular problems spinal muscular atrophy spina bifida Guillain-Barre syndrome myasthenia gravis muscular dystrophy myotonic dystrophy
How to differentiate between clinical shock and dehydration?
pale and cold extremities
prolonged capillary refill time
Tachycardia, tachypnoea, reduce skin turgor, urine output are present in both.
Differences between early compensated and late decompensated shock?
Late: Hypotensive, bradycardic, kussmaul breathing, blue extremities and absent (as opposed to reduced) urine output
NB any sign of shock (early/late) requires urgent fluid resus not just maintenance fluids.
Disorders that have a trinucelotide repeat expansion
Fragile X and Myotonic Dystrophy were the first disorders discovered to have this inheritance pattern, Huntington, Friedrich’s and cerebellar ataxia are other disorders
Myotonic Dystrophy
Type 1: Mutation in DMPK gene on chromosome 19. CTG trinucleotide repeat. This codes for Myotonic Dystrophy Protein kinase, which inhibits myosin phosphatase which is responsible for muscle contraction
Type 2: Mutation in CNBP gene on Chromosome 3. CCTG contains a tetranucleotide repeat. CNBP codes for cellular nucleic acid binding protein which controls the expression of genes in the heart and muscle.
In both types there is repeat expansion due to slipped mispairing by DNA Polymerase. If there is more than double (t1) or triple (t2) number of repeats than normal it can manifest into muscular dystrophy. If there are increased repeats, the pt is in a pre-mutation state of muscular dystrophy.
Myotonia = sustained muscle contraction i.e. difficulty relaxing e.g. difficulty letting go of a handshake.
Type 1: Can either present congenitally as floppy baby, with respiratory difficulty and muscle weakness, quite fatal. Or can present in adulthood with muscle weakness in the face (droopy eyelids and hollow cheeks), arms and legs.
Type 2: Presents with adult onset muscular weakness and is typically milder. There is proximal muscle weakness and weakness in shoulder/arms (meaning that there is a difficulty lifting objects, walking up and down the stairs).
Other: there is an association with cataracts, insulin resistance and cardiomyopathies. There is an association with GC tumours and so be wary of testicular swelling in kids.
Ix: muscle biopsy, EEMG, genetic testing.
Mx: Symptomatic
Neurofibromatosis
Type 1 and Type 2
Type 1=
COFFES (2 or more signs needed for diagnosis)
C = Cutaneous manifestations (Neurofibroma (nodules under the skin that are nerve tumours) and cafe au lait [6+ that are >5mm if pre pubescent and >15mm if post puberty])
O = Optic glioma
F= Freckles in axillary
F= First degree relative
E= Eye spots (lisch nodules)
S= Skull lesions (Boney lesions from sphenoid dysplasia)
Type 2 =
Less common
The main manifestation of the condition is the development of bilateral benign brain tumors in the nerve sheath of the cranial nerve VIII, which is the “auditory-vestibular nerve” that transmits sensory information from the inner ear to the brain (schwannoma). Besides, other benign brain and spinal tumors occur
Marfan’s
Car = Autosomal Dominant
15 necklace = Chromosome 15 fibrillin 1 (FBN1) mutation
Skeletal features: Arachnodactyl, pectus excavatum, scoliosis,
Tall, long fingers, (same as homocysteinuria) but lens subluxed UPWARDS and OUTWARDS (different to homocysteinuria - lens is downwards and inwards)
Hat - Mitral PROLAPSE Sx (mid systolic click, late systolic murmur)
Tree - Aortic Dissection is most common cause of mortality
Mx: Beta blockers and losartan to slow the dilatation
Measles
RNA paramyxovirus
spread by droplets
infective from prodrome until 4 days after rash starts
incubation period = 10-14 days
prodrome: irritable, conjunctivitis, fever
Koplik spots (before rash): white spots (‘grain of salt’) on buccal mucosa
rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent
Ix: IgM
Mx: Supportive, inform PH. If not immunised you can give MMR within 72 hours of exposure.
Complications
otitis media: the most common complication
pneumonia: the most common cause of death
encephalitis: typically occurs 1-2 weeks following the onset of the illness)
subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness
febrile convulsions
keratoconjunctivitis, corneal ulceration
diarrhoea
increased incidence of appendicitis
myocarditis
Scarlet fever cx
Scarlet fever is usually a mild illness but may be complicated by:
otitis media: the most common complication
rheumatic fever: typically occurs 20 days after infection
acute glomerulonephritis: typically occurs 10 days after infection
invasive complications (e.g. bacteraemia, meningitis, necrotizing fasciitis) are rare but may present acutely with life-threatening illness
Scarlet fever mx and ix
Diagnosis
a throat swab is normally taken but antibiotic treatment should be commenced immediately, rather than waiting for the results
Management
oral penicillin V for 10 days
patients who have a penicillin allergy should be given azithromycin
children can return to school 24 hours after commencing antibiotics
scarlet fever is a notifiable disease
Parvovirus
The illness may consist of a mild feverish illness which is hardly noticeable. However, in others there is a noticeable rash which appears after a few days. The rose-red rash makes the cheeks appear bright red, hence the name ‘slapped cheek syndrome’. The rash may spread to the rest of the body but unlike many other rashes, it only rarely involves the palms and soles.
The child begins to feel better as the rash appears and the rash usually peaks after a week and then fades. The rash is unusual in that for some months afterwards, a warm bath, sunlight, heat or fever will trigger a recurrence of the bright red cheeks and the rash itself. Most children recover and need no specific treatment. School exclusion is unnecessary as the child is not infectious once the rash emerges. In adults, the virus may cause acute arthritis.
Consent in children
The family law reform act of 1969 states that ‘those over 16 can consent to treatment, but cannot refuse treatment under 18 unless there is one consenting parent, even if the other disagrees’.
at 16 years or older a young person can be treated as an adult and can be presumed to have capacity to decide
under the age of 16 years children may have capacity to decide, depending on their ability to understand what is involved
where a competent child refuses treatment, a person with parental responsibility or the court may authorise investigation or treatment which is in the child’s best interests*
Gillick competence is for contraception only
Cow’s milk allergy
Cow’s milk protein intolerance/allergy (CMPI/CMPA) occurs in around 3-6% of all children and typically presents in the first 3 months of life in formula-fed infants, although rarely it is seen in exclusively breastfed infants.
Both immediate (IgE mediated) and delayed (non-IgE mediated) reactions are seen. The term CMPA is usually used for immediate reactions and CMPI for mild-moderate delayed reactions.
Features regurgitation and vomiting diarrhoea urticaria, atopic eczema 'colic' symptoms: irritability, crying wheeze, chronic cough rarely angioedema and anaphylaxis may occur
Diagnosis is often clinical (e.g. improvement with cow’s milk protein elimination).
Ix: skin prick/patch testing, total IgE and specific IgE (RAST) for cow’s milk protein
Management
If severe (e.g. failure to thrive) refer to a paediatrician.
If formula fed:
eHF - extensive hydrolysed formula
AAF - amino acid-based formula infants with severe CMPA or if no response to eHF
If breastfed: eliminate CM and continue breastfeeding, consider prescribing calcium supplements for breastfeeding mothers whose babies have, or are suspected to have, CMPI, to prevent deficiency
CMPI usually resolves in most children by age of 5
