Paediatrics & Child Health - part 8 doc

Severe stenosis: may present with exercise intolerance, angina on exertion,and heart failure.. E: Mild to moderate stenosis: child is usually acyanotic with a right lar heave þ= systolic

sunlight, trauma, medications (b-blockers, antimalarials).

A/R: HLA types: CW6, B13, and B17

E: Incidence: 1–3% of the world’s population

Age of onset: < 2 years: 2%, < 5 years: 6.5%, < 10 years: 10%, < 15 years:27%, < 20 years: 37%

Race: commoner in Caucasians F : M ¼ 2 :1 (Norwegian psoriasis study)

H: Infants: intractable nappy rash

Children: itching or occasionally tender skin

Auspitz phenomenon: pinpoint bleeding with removal of scales

Koebner phenomenon: skin lesions develop at the site of trauma/scars

E: Well-demarcated, erythematous, scaling papules and plaques Children monly have facial lesions Nail pitting and onycholysis is rare in children

com-Guttate psoriasis: commonest presentation in children 5–12 years old;small, drop-like plaques over trunk, limbs; occurs post streptococcal infection(tonsillitis), usually resolves over 3–4 months

Plaque psoriasis: less common; well-defined, disc-shaped plaques on elbows,knees, scalp hair margin, or sacrum, covered by silvery scales

Napkin psoriasis: well-defined eruption in nappy area of infants

Generalised pustular psoriasis (rare): acute development of sheets ofyellow pustules on erythematous background with associated fever

P: Rapid epidermal proliferation (20 normal), possibly driven by cytokines leased by T lymphocytes in the dermis, and associated accelerated upwardmigration of immature keratinocytes

re-I: Majority do not need investigating as psoriasis is a clinical diagnosis

Guttate psoriasis: ASOT, throat swab

Nail involvement: analyse nail clippings to exclude fungal infection

M: Topical: emollients for moisturisation, coal tar (#DNA synthesis), dithranol(anti-mitotic, irritates normal skin, stains), topical steroids (moderately potent,e.g eumovate), vitamin D3 analogue (e.g calcipotriol, inhibits cell prolifer-ation and stimulates keratinocyte differentiation)

UV Light: oral PUVA or UVB (for widespread thin lesions or guttate psoriasis).Systemic: in severe cases use methotrexate (anti-inflammatory and immunemodulatory, risk of liver cirrhosis, teratogenic), cyclosporin (immunosuppres-

sant), retinoids (for pustular psoriasis), etanercept (anti-TNFa; especially for

psoriatic arthritis)

Advice: avoid exacerbating factors

C: Seronegative arthritides:

(1) Distal asymmetrical oligoarthritis (DIP joints)

(2) Dactylitis (IP arthritis and flexor tenosynovitis)

(3) Rheumatoid arthritis–like (symmetrical polyarthritis)

(4) Arthritis mutilans (telescoping of the digits)

(5) Ankylosing spondylitis

Other complications: anterior uveitis, erythroderma

143

develop-A/R: Other congenital heart defects; ASD, VSD, PDA.

E: Represents 8–12% of all congenital heart defects Occurs in as many as 50% ofall patients with other congenital cardiac lesions M ¼ F

H: Mild stenosis: most children are asymptomatic in infancy and childhood.Moderate stenosis: dyspnoea and fatigue appear as severity and decompen-sation increases

Severe stenosis: may present with exercise intolerance, angina on exertion,and heart failure Rarely, severe stenosis may present with cyanosis due to right

! left shunting through the foramen ovale or an associated ASD

E: Mild to moderate stenosis: child is usually acyanotic with a right lar heave þ=
systolic thrill, S1 is followed by a click and S2 is delayed, systolicmurmur is heard loudest at the left upper sternal border, radiating to theback; the severity of stenosis is directly related to intensity and duration ofthe murmur

ventricu-Severe stenosis: cyanosis, signs of heart failure with tricuspid insufficiency;giant ‘a’ waves in JVP, hepatomegaly, and a pulsatile liver

P: Fusion of the leaflet commissures results in a thickened and domed ance to the valve

appear-I: CXR: normal heart size, post-stenotic dilatation of PA, #pulmonary bloodflow May show signs of CHF with right ventricular and atrial enlargement.ECG: normal in mild stenosis, but in severe stenosis may show right axis devi-ation, RVH, and signs of right heart strain

Doppler echo: diagnostic and determines severity of stenosis

M: Valvular pulmonary stenosis: management determined by the Dopplergradient:

(1) Mild: no treatment, follow-up screening examination and ECG for 3–5years

(2) Moderate: depends on whether symptomatic, and weighing up of risk/benefit ratio

(3) Severe: proceed to cardiac catheterisation þ=
balloon valvuloplasty orvalvotomy

Infundibular and supravalvular pulmonary stenosis: if severe, requireoperative and invasive surgical intervention

C: RVH and CHF in severe pulmonary stenosis

P: Mild valvular pulmonary stenosis usually does not progress, but the moderate

to severe disease does

Following balloon or surgical valvotomy, the prognosis is excellent RVH gresses and the condition does not recur Life expectancy is similar to the144

concord-There is speculation on the role of gastrin and myenteric plexus abnormalities.

A/R: Family history: 5–10% of infants have previously affected parents, first-bornchildren

E: 1/200 live births; the most common cause of intestinal obstruction in infancy,commoner in Caucasians

(2) Persistently hungry following projectile vomiting

P: Marked hypertrophy and hyperplasia of the 2 (circular and longitudinal) cular layers of the pylorus occurs, which ! narrowing of the gastric antrum.The pyloric canal becomes lengthened and the whole pylorus becomesthickened The mucosa is usually oedematous and thickened In advancedcases, the stomach is markedly dilated

mus-I: Bloods: U&E for hypochloraemic hypokalaemic alkalosis due to vomiting;

"pH, # Kþ, # Cl
, # Na2þ, " HCO

3, "urea May have mild, unconjugatedhyperbilirubinaemia

USS abdomen: performed in most cases where pyloric stenosis is suspected

M: Pre-op: fluid resuscitation and correction of electrolyte imbalance NG tube isrequired to relieve gastric contents

Ramstedt pyloromyotomy: an incision is made in the pyloric canal todivide the hypertrophied muscle fibres down to, but not through, the pyloricmucosa

C: Surgical intervention prevents complications without which dehydration andelectrolyte disturbance are usually fatal

P: Excellent following surgery Surgery carries < 1% mortality rate Feeding is

145

Recurrent abdominal pain

D: Abdominal pain sufficient to interrupt normal activities

A: Functional abdominal pain: 90% of children who experience recurrent dominal pain have no structural or mucosal abnormality in the GI tract.Specific GI disorders: 10% of recurrent abdominal pain is due to IBS, non-ulcer dyspepsia, and abdominal migraine

ab-A/R: Stress at home/school, anxiety

E: Incidence: 10% of school-age children

H: Functional abdominal pain: pain is characteristically around the umbilicus,does not wake the child at night, and is not associated with food, feeding, orbowel habit The child is otherwise well

IBS: pain is often worse before and relieved by defaecation, stools haveexcess mucous; children experience bloating, sensation of incomplete defaeca-tion, and constipation

Non-ulcer dyspepsia: epigastric pain, post-prandial vomiting, early satiety,and acid reflux

Abdominal migraine: pain is paroxysmal, stereotypic, and may be associatedwith facial pallor þ=
headache (throbbing, unilateral, aura)

E: There may be no findings or mild generalised abdominal tenderness

P: IBS: abnormal contractions of the intestines, which are modulated by ating levels of stress and anxiety

fluctu-Non-ulcer dyspepsia: abnormal gastric motility

Abdominal migraine: classical cranial migraine is associated with abdominalpain, in children the abdominal pain can predominate

I: Investigations should only be performed if clinically indicated.Urine analysis and culture may be performed to exclude a UTI

M: Education: the diagnosis of functional abdominal pain should be made as apositive diagnosis rather than a diagnosis of exclusion, or the child will beexposed to unnecessary investigations

Medical: famotidine (H2-blocker), pizotifen (antihistamine and serotonin tagonist), and peppermint oil enteric-coated capsules have been shown to

an-# measured pain outcomes of recurrent abdominal pain when compared withothers in control groups There was greater improvement when therapy wastargeted to the specific GI disorder (dyspepsia, abdominal migraine, IBS).Using drugs can, however, risk somatising a functional, usually self-limiting,disorder

Behavioural: CBT and biofeedback have been shown to be effective in creasing pain scores The behavioural interventions seem to have a generalpositive effect on children with true functional abdominal pain

de-Dietary: studies that have evaluated dietary interventions have had conflictingresults in the case of fibre, or showed no efficacy in the case of lactose avoid-ance

C: Functional abdominal pain may become a strategy of school avoidance If nottackled effectively, this may affect the child’s school performance, and !further behavioural problems

P: 50% of children affected with functional abdominal pain resolve rapidly In25% the pain resolves in a few months, and in 25% the symptoms continue146

Renal failure, acute (ARF)

D: A significant deterioration in renal function occurring over hours or days,resulting in "plasma urea, creatinine, and oliguria Complete recovery of renalfunction usually occurs within days/weeks

(2) Acute GN (see chapter)

(3) Acute interstitial nephritis (infection, drugs; NSAIDs, frusemide, penicillin).(4) Small/large vessel obstruction (renal artery/vein thrombosis, vasculitis, HUS,TTP)

Post-renal (obstructive):

(1) Neuropathic bladder; may be acute in transverse myelitis, spinal trauma

(2) Stones (bilateral pelviureteric junction or ureteral)

(3) Urethral prolapse of bladder ureterocele

A/R: Acute illnesses and multiorgan failure

E: 0.8/100 000 children

H: Vomiting, anorexia, oliguria, convulsions, previous sore throat and fever streptococcal GN), bloody diarrhoea and progressive pallor (HUS), drug his-tory

(post-E: Assess intravascular volume status: volume depleted (cool peripheries, cardia, postural hypotension) or overloaded Is patient septic? Is patient ob-structed? Examine abdomen for palpable bladder

tachy-P: Acute tubular necrosis:

Macro: enlarged kidneys with pale cortex Micro: swelling and necrosis of thetubular cells, interstitial oedema with macrophage and plasma cell infiltration

I: Bloods: #Hb (hypovolaemia/haemorrhage), "WCC, "CRP, blood cultures(sepsis), "urea, "creatinine, " Kþ, "phosphate, # Ca2þ, # Mg2þ, LFTs, venouscapillary blood gas, clotting (DIC), ASOT (post-streptococcal GN)

Blood film: HUS/TTP (RBC fragmentation)

Urine: Urinalysis for blood, protein (GN), glucose (interstitial nephritis), scopy for casts (GN), urine Naþ, urea, creatinine, osmolality to differentiatebetween pre-renal and intrinsic renal failure

micro-ECG: signs of hyperkalaemia; tall tented T waves ! small or absent P waves

!"P–R interval ! widened QRS complex ! sine wave pattern ! asystole

CXR: signs of pulmonary oedema

Renal USS: in ARF, kidneys appear normal or increased in size and city, may detect stones or clot in RVT

echogeni-Renal biopsy: if diagnosis has not been determined

M: Resuscitate: especially in pre-renal causes of ATN

Monitor: daily U&E, temperature, PR, RR, BP, O2 saturation, hourly urineoutput, CVP, daily weights

Treat cause: avoid potential causative drugs, post-renal causes; catheters,stents, nephrostomy or surgery, hypovolaemia; fluids, sepsis; antibiotics

Nutrition: high-calorie intake, with enteral/parenteral nutrition if oral intake

147

Renal failure, acute (ARF) continued

Dialysis: indications for acute dialysis:

(1) Severe extracellular fluid volume overload; "BP, pulmonary oedema notresponding to diuretics

(2) Severe "Kþ; not responding to medical treatment

(3) Severe systematic uraemia

(4) Severe metabolic acidosis, not controllable with IV sodium bicarbonate.(5) Removal of toxins (drugs, poisons)

C: Heart failure and pulmonary oedema (volume overload), GI bleeding (gastriculceration, gastritis, and platelet dysfunction), muscle wasting due to hyperca-tabolic state, uraemic pericarditis/encephalopathy

P: Depends on the causative factor Recovery of renal function following ARF ismost likely following pre-renal causes, HUS, ATN, acute interstitial nephritis,

or uric acid nephropathy

148

Renal failure, chronic (CRF)D: Characterised by #GFR, persistently "urea and "creatinine concentration.

A: Age <<5 years: congenital abnormalities (hypoplasia, dysplasia, obstruction(posterior urethral valve), malformations)

I: Bloods: #Hb, MCV (usually normocytic) # Naþ, " Kþ, "urea, "creatinine,

# Ca2þ, "phosphate, "ALP, "PTH (28 hyperparathyroidism)

Urine: 24-h collection for protein and creatinine clearance

X-rays: for signs of osteomalacia and hyperparathyroidism

Renal USS: for anatomical/hereditary abnormalities, measure size (smallshrunken kidneys consistent with CRF), exclude obstruction/stones

Renal biopsy: for changes specific to the underlying disease, contraindicated

(3) Ca2þmaintenance: 1-hydroxylated vitamin D analogues, e.g alfacalcidol

(4) Diet: high-energy intake, restrict Kþin hyperkalaemia or acidosis, restriction

of phosphate intake combined with use of phosphate binders to prevent 28hyperparathyroidism

(5) Drugs: avoid nephrotoxic drugs, adjust doses of other drugs, e.g frusemide

in oedema

Continuous ambulatory peritoneal dialysis: dialysate is introduced andexchanged through a catheter, inserted via an SC tunnel into the peritoneum.Preferred method in children

Haemodialysis: blood is removed via an arteriovenous fistula surgically structed in the forearm to provide high flow Uraemic toxins are removed bydiffusion across a semipermeable membrane in an extracorporeal circuit

con-Transplantation: in end-stage renal failure Requires long-term pressants to # rejection

immunosup-C: Haematological: anaemia, abnormal platelet activity (bruising, epistaxis)

Cardiovascular: accelerated atherosclerosis, "BP, and pericarditis

Neurological: peripheral and autonomic neuropathy, proximal myopathy

Renal osteodystrophy: osteoporosis, osteomalacia, 28/38 hyperparathyroidism.Endocrine: amenorrhoea

149

Renal failure, chronic (CRF) continued

Haemodialysis:

(1) Acute: hypotension due to excessive removal of extracellular fluid.(2) Long-term: atherosclerosis, sepsis (28 peritonitis with Staph aureus infec-tion)

(3) Amyloidosis: ! periarticular deposition, arthralgia (e.g shoulder) andcarpal tunnel syndrome

Transplantation//immunosuppression: opportunistic infections (e.g.Pneumocystis carinii), malignancies (lymphomas and skin), and side-effects ofimmunosuppressant drugs

P: Depends on complications Timely dialysis/transplantation improves survival.150

Respiratory distress syndrome (RDS)

D: Respiratory compromise in the newborn preterm infant 28 to surfactant ciency

defi-A: The clinical syndrome of RDS arises from the interplay of a number of factors: Small lung volumes due to immaturity

Surfactant deficiency that ! high alveolar surface tension, alveolar lapse, and intrapleural right ! left shunting

col- 188 surfactant deficiency: due to prematurity and promoted by hypoxia,acidosis, hypothermia, and hypotension during the delivery

288 surfactant deficiency: intrapartum asphyxia, pulmonary infections orhaemorrhage, meconium aspiration, pneumonia

Soft thoracic cage means that as the neonate attempts to generate a largenegative intrathoracic pressure the ribs and sternum ‘cave in’ and the ab-dominal contents are displaced downwards This type of breathing is inef-fective and ! classical ‘see-saw’ breathing

A/R: Preterm delivery, maternal DM, Caesarean section delivery infants, born twins, family history

second-E: 50% of infants born at 28–32 weeks gestation develop RDS The majority ofinfants < 28 weeks have RDS

H&

E:

Progressive signs of respiratory distress: tachypnoea, grunting ation against partially closed glottis), subcostal and intercostal recession, nasalflaring, and with extremely premature infants apnoea þ=
hypothermia maydevelop, and cyanosis

(expir-P: Macroscopic: lungs appear airless and ruddy (liver-like)

Microscopic: diffuse atelectasis of the distal airspaces with distension ofsome of the distal airways and perilymphatic areas

I: ABG:

(1) Respiratory acidosis due to alveolar atelectasis þ=
overdistension of minal airways

ter-(2) Metabolic acidosis due to lactic acidosis 28 to poor tissue perfusion

(3) Hypoxia due to right ! left shunting

CXR: bilateral diffuse reticular granular or ground glass appearance, airbronchograms, and poor lung expansion

Echo: to determine presence of PDA

M: Prevention:

Identification of at-risk infants, neonatologist/NICU early involvement

Amniocentesis for estimation of foetal lung maturity by myelin ratio and presence of phosphatidylglycerol in at-risk infants

lecithin/sphingo- The prudent use of antenatal steroids stimulates foetal surfactant tion and is used when preterm delivery is anticipated

produc-Treatment:

Surfactant replacement therapy; reduces mortality from RDS by 40%

Correction of hypoglycaemia, hypothermia, and electrolyte imbalances

Oxygen and CPAP via nasal cannulae, ventilation either conventional oroscillatory

Prophylactic antibiotics to prevent respiratory infections

C: Acute: alveolar rupture ! pneumothorax, intracranial haemorrhage, ventricular leucomalacia (ischaemic necrosis of periventricular white matter),PDA, pulmonary haemorrhage, NEC, or GI perforation

peri-Chronic: CLD of prematurity, ROP, neurological impairment

P: Previously extremely poor (60% mortality) but improving with antenatal

ster-151

Retinopathy of prematurity (ROP)

D: Serious vasoproliferative disorder affecting extremely preterm infants

A: Infants at highest risk for ROP are those with the lowest birth weight andgestational age although many other factors are associated with "risk:(1) Severity of general illness

(2) Prolonged exposure to high concentrations of supplemental oxygen.(3) Persistent acidosis, period of mechanical ventilation

H: All neonates who are at risk should be screened: gestational age < 32 weeks,birth weight < 1500 g Screening begins at 4 weeks of age and continues untilthe retina is seen to be fully vascularised

E: Should be performed by an experienced ophthalmologist International fication system for ROP uses disease zones 1–3 which measures extent ofretina involved, stage of disease, which measures severity and ‘plus’ disease(tortuous dilated retinal vessels) which implies active progressive disease

classi-P: The retinal vasculature begins to form in the 16th week of gestation Retinalvessels grow out of the optic disc as a wave of mesenchymal spindle cells Inpreterm infants normal retinal vascular maturation is interrupted The bloodvessels constrict and atrophy, which disrupts the blood supply to the retinaand causes ischaemia Angiogenic factors (e.g vascular endothelial growthfactor) are released from the mesenchymal spindle cells and the ischaemicretina and ! new vessel proliferation New vessels are tortuous and fragileand may haemorrhage, which results in fibrosis and subsequently retinaldetachment

I: Diagnosis is based on findings of clinical examination

M: Prevention: likelihood is reduced with careful control of pO2in the lated child and use of O

venti-2concentrations of < 40%

Neonatal screening: studies have shown that ablative therapy to destroythe avascular areas of the retina in threshold disease improves outcome.Ablative surgery:

Cryotherapy (freezing): requires general or local anaesthesia

Complications: intraocular haemorrhage, conjunctival haematoma or ation, and bradycardia

lacer-Laser therapy: preferred option to cryotherapy as the ocular tissues are lesstraumatised, general anaesthesia is avoided, and there are fewer complications.Complications: cataracts, intraocular haemorrhages

C: Severe visual impairment, myopia, amblyopia, and strabismus

P: Patients should receive yearly ophthalmology follow-up as the long-term152

Rheumatic fever

D: A systemic inflammatory disorder affecting the heart, joints, CNS, skin, and SCtissue, characterised by an exudative and proliferative inflammatory lesion ofthe connective tissue

A: Follows 0.3% of group A b-streptococcal infection usually of the URT.

A//R: Malnutrition, overcrowding, socio-economically disadvantaged groups

E: Still common in developing countries; however, has decreased in developedcountries due to "use of penicillin Peak age: 5–15 years M ¼ F

H&

E:

Rheumatic fever occurs  20 days after streptococcal throat infection

Diagnosed by modified Duckett Jones criteria (2 major or 1 major and 2 minor):Major:

(1) Carditis

(2) Migratory polyarthritis

(3) Erythema marginatum (serpiginous

flat, nonscarring painless rash)

(4) SC nodules

(5) Sydenham’s chorea (rapid

uncoordinated jerky movements

primarily of hands, feet and face)

Minor:

(1) Fever(2) Arthralgia(3) Previous rheumatic fever

or carditis(4) Positive ESR/CRP(5) Leucocytosis(6) Prolonged PR interval

Presentation can be of sudden onset, typically beginning with a polyarthritis2–6 weeks after streptococcal pharyngitis, and is usually characterised by pyr-exia and toxicity

Presentation may be of insidious onset with mild carditis, usually as a result of asub-clinical infection

P: Joints: non-specific oedema and hyperaemia of inflamed synovial membranes.Cardiac: acute interstitial valvulitis causing valvular oedema, thickening,fusion, and retraction of leaflets and cusps This results in valvular stenosis orregurgitation Aschoff bodies are found in the myocardium

Skin: nodule biopsies resemble Aschoff bodies

I: No investigation is pathognomonic; diagnosis is confirmed using the modifiedDuckett Jones criteria

Bloods: "ESR/CRP, "WCC, ASOT Throat swab: MC+S

Local inflammation: "WCC with negative cultures in synovial fluid (usuallyclear/yellow)

ECG: PR prolongation in acute carditis

ECHO: mitral regurgitation, myocarditis, pericarditis

M: Treat infection: IV pencillin or cephalosporins

Arthritis: analgesics such as codeine or NSAIDs in mild cases, aggressive use

of anti-inflammatory drugs may be required in severe cases

Carditis: NSAIDs to suppress inflammation In severe carditis with heart ure corticosteroids (prednisolone) may be started

fail-Antistreptococcal prophylaxis: penicillin V orally for 25 years to preventrecurrence

C: Repeated streptococcal infections, damage to the heart valves (especially mitraland aortic stenosis), endocarditis, heart failure, arrhythmias, and pericarditis

P: Duration of illness: in 75% of cases the acute attack lasts 6 weeks, 90%have resolved in 12 weeks, and only 5% of patients have symptoms that per-sist for > 6 months

Risk factors for CRHD: include the severity of the initial carditis, the presence

or absence of recurrences, and the amount of time since the episode of atic fever

rheum-Incidence of CRHD: at 10 years after initial presentation, incidence of CRHD is34% in patients without recurrences but 60% in patients with recurrent rheum-

153