Paediatrics Pt. 2 Flashcards

Question

How is encephalitis managed?

Answer 1

- Proven/suspected HSV encephalitis should be treated with IV aciclovir for 3 weeks, as relapses may occur after shorter courses - If untreated, the mortality rate is > 70% and survivors usually have serious neurological sequelae

Answer 2

- This syndrome can be caused by: - Toxin-producing S. aureus - Group A streptococci - It is characterised by - Fever > 39 degrees - Hypotension - Diffuse erythematous, macular rash - The toxin can be released from an infection at any site (including small abrasions and burns which look minor) - The toxin acts as a SUPERANTIGEN and can cause organ dysfunction - Mucositis (conjunctiva, oral mucosa, genital mucosa) - Gastrointestinal dysfunction (vomiting/diarrhoea) - Renal impairment - Liver impairment - Clotting abnormalities and thrombocytopaenia - CNS (altered consciousness) - Intensive care support is required to manage patients in shock - Areas of infection should be surgically debrided

Answer 3

- 3rd generation cephalosporin (e.g. ceftriaxone) - Clindamycin - This acts on the bacterial ribosome to switch off toxin production - IVIG - May be given to neutralise the circulating toxin

Answer 4

- This is produced by < 2% of S. aureus strains - PVL-producing S. aureus causes recurrent skin and soft tissue infections - They can also cause necrotising fasciitis and a necrotising haemorrhagic pneumonia following an influenza-like illness - These both carry a high mortality rate - In children, the procoagulant state induced by the toxin can cause venous thrombosis

Answer 5

- This is a RARE, severe subcutaneous infection - The area involved may enlarge rapidly, leaving poorly perfused necrotic areas of tissue at the centre - It is accompanied by severe pain and systemic illness and usually required intensive care - Invading organisms: - Staphylococcus aureus - Group A streptococcus - NOTE: these can exist with or without a synergistic anaerobic organism (i.e. it can be a mixed infection)

Answer 6

- SURGICAL EMERGENCY - Debridement of necrotic tissue - IV fluids - Empirical IV antibiotics (vancomycin, linezolid, daptomycin, tedizolid phosphate, tazocin, meropenem, imipenem/cilastatin, ertapenem) - Any clinical suspicion of necrotising fasciitis requires urgent surgical consultation - IVIG may also be given

Answer 7

- This is a particularly DANGEROUS infection because it can potentially kill previously healthy children within hours - Meningococcal infection has the lowest risk of long-term neurological sequelae with most survivors recovering fully - PURPURIC RASH in meningococcal septicaemia - The rash is characteristically non-blanching on palpation, irregular in size and outline, and may have a necrotic centre - ANY FEBRILE CHILD with a purpuric rash should be treated IMMEDIATELY with IM penicillin or IV 3rd generation cephalosporin before urgent admission to hospital - NOTE: after the inclusion of the meningococcus C vaccine in the UK, most cases of meningococcal septicaemia are caused by group B meningococci - FUTURE: polysaccharide conjugate vaccines have been developed against group A, B and C meningococci so the incidence of meningococcal disease should continue to decline

Answer 8

- Streptococcus pneumoniae in the nasopharynx of healthy children - This can be transmitted to other individuals via respiratory droplets - Streptococcus pneumoniae can cause: - Pharyngitis - Otitis media - Conjunctivitis - Sinusitis - Invasive disease (pneumonia, bacterial sepsis, meningitis) - This mainly occurs in young infants as their immune system is weak against encapsulated pathogens - It carries a high morbidity and mortality - NOTE: following the inclusion of the 13-valent pneumococcal vaccine in the UK, the incidence of invasive pneumococcal disease has declined - Children who are at risk (e.g. due to hyposplenism) should be given daily prophylactic penicillin to prevent infection

Answer 9

- Hib used to be an important cause of systemic illness in children (e.g. otitis media, pneumonia, meningitis) - However, immunisation has been highly effective and Hib now rarely causes systemic disease

Answer 10

- Usually caused by direct invasion of the organisms - Can release toxins which act as superantigens - Normal antigens will stimulate only a small subset of T cells, which have a specific antigen receptor - Whereas, superantigens bind to part of the TCR which is shared by many T cells - This results in massive T cell proliferation and cytokine release - Some disease that follow streptococcal infections (e.g. post-streptococcal glomerulonephritis and rheumatic fever) are immune-mediated - Impetigo - Highly contagious, staphylococcal or streptococcal skin infection - It most commonly occurs in infants and young children - More common in children with pre-existing skin disease (e.g. eczema) - Lesions are usually found on the face, neck and hands - They begin as erythematous macules which becomes vesicular/pustular or even bullous - Rupture of the vesicles with exudation of fluid leads to the honey-coloured crusted lesions - Infection readily spreads to adjacent areas and other parts of the body by autoinoculation of the infected exudate

Answer 11

- Advice - Leaflets from British Association of Dermatologists (BAD) - Reassure that impetigo usually heals without any scarring - Hygiene is important: wash areas with soapy water, wash hands after touching lesions, avoid scratching affected areas and keep nails short, avoid sharing towels/bathwater etc. - Children should avoid school until the lesions are dry and scabbed over - Follow-up if no improvement after 7 days - Medical Treatment - Localised Infection = topical fusidic acid (3-4/day for 7 days) - Extensive Infection = oral flucloxacillin (4/day for 7 days) - Clarithromycin can be used if penicillin allergy - Bullous Infection = oral flucloxacillin or clarithromycin/erythromycin - Review diagnosis - Check compliance with treatment and hygiene measures - Take a swab - Consider oral antibiotics if fusidic acid was initially used

Answer 12

- S. aureus infections of hair follicles or sweat glands - TREATMENT: systemic antibiotics and (occasionally) surgical incision - Recurrent boils may occur due to persistent nasal carriage in the child or the family acting as a reservoir for reinfection - RARELY, recurrent boils can be a feature of underlying immunodeficiency

Answer 13

- Fever with erythema, tenderness and oedema of the eyelid or other skin adjacent to the eye - Almost always unilateral - May follow trauma to the skin - In older children, it can spread from a paranasal sinus infection or dental abscess

Answer 14

- PROMPTLY IV antibiotics (e.g. high-dose ceftriaxone) - To prevent orbital cellulitis - Incision and drainage of peri-ocular abscess may be required

Answer 15

- Proptosis - Painful or limited ocular movement with/without reduced visual acuity - Can be complicated by abscess formation, meningitis or cavernous sinus thrombosis

Answer 16

- Caused by an exfoliative staphylococcal toxin - It causes the separation of the epidermal skin through the granular cell layers - It mainly affects infants and young children

Answer 17

- Fever - Malaise - Purulent, crusting and localised infection around the eyes, nose and mouth - Erythema and tenderness of the skin - Nikolsky Sign: areas of epidermis will separate on gentle pressure, leaving denuded areas of skin - NOTE: these areas of skin tend to heal without scarring

Answer 18

- IV antibiotics (flucloxacillin) - Analgesia - Monitoring hydration and fluid balance

Answer 19

- A fever and a rash - Incubation periods of viral infections can vary from 24-48 hours for viral gastroenteritis, to about 2 weeks for chickenpox - For some diseases, like HIV, the time between exposure and the development of symptomatic illness can be many years - The infectious period characteristically begins 1-2 days before the rash appears and (for the purposes of nursery/school exclusion) lasts until the rash has resolved and the lesions have dried up

Answer 20

- HSV1 - HSV2 - VZV - CMV - EBV - HHV-6 - HHV-7 - HHV-8

Answer 21

- After primary infection, latency is established - Virus stays in the host for a long time - This can be reactivated after certain stimuli

Answer 22

- HSV1 = lip and skin lesions - HSV2 = genital lesions - NOTE: both viruses an cause both types of disease

Answer 23

- Paracetamol or ibuprofen for pain and fever - Aciclovir (DNA polymerase inhibitor) may be considered - IMPORTANT: most herpes simplex infections are asymptomatic

Answer 24

- MOST COMMON form of primary HSV in children - Usually occurs in 10 months to 3 years of age - Presents with vesicular lesions on the lips, gums and anterior surfaces of the tongue and hard palate - They usually progress to extensive, painful ulceration and bleeding - It is often accompanied by a high fever - The illness can persist for up to 2 weeks - Eating and drinking becomes painful, resulting in dehydration

Answer 25

- Symptomatic | - Severe cases may require IV fluids and aciclovir

Answer 26

- Mucocutaneous junctions and damaged skin are particularly prone to HSV infection - Cold sores are recurrent HSV lesions on the gingival/lip margin - Eczema herpeticum - Widespread vesicular lesions develop on eczematous skin - This can be complicated by secondary bacterial infection, which can then, in turn, result in septicaemia - Herpetic Whitlows - Painful, erythematous and oedematous white pustules on the site of broken skin (usually on fingers) - It is spread by autoinoculation from gingivostomatitis

Answer 27

- Blepharitis or conjunctivitis - It an involve the cornea and cause dendritic ulceration - This can result in corneal scarring and loss of vision - ANY herpetic lesions near or in the eye require urgent ophthalmic assessment

Answer 28

- May be focal (e.g. affecting the skin, eyes or encephalitis) - May be disseminated - High morbidity and mortality

Answer 29

- May be severe - Cutaneous lesions can spread to involve adjacent sites (e.g. oesophagitis, proctitis) - Pneumonia and disseminated infections involving multiple organs are major complications

Answer 30

- Secondary Bacterial Infection - Mainly with staphylococci and group A streptococci - Can lead to toxic shock syndrome and necrotising fasciitis - Should be considered when there is onset of a new fever or persistent high fever after the first few days - Encephalitis - May be generalised - Usually occurs early in the illness - GOOD prognosis (as opposed to HSV encephalitis) - Characteristically causes a VZV-associated cerebellitis - The child becomes ataxic with cerebellar signs - This usually occurs around 1 week after the onset of the rash - Usually resolves within a month - Purpura Fulminans - Consequence of vasculitis in the skin and subcutaneous tissues - Best known in relation to meningococcal disease (purpuric rash) - Can lead to the loss of large areas of skin by necrosis - Rarely, after VZV infection, antiviral antibodies can cross-react and inactivate inhibitory coagulation factors (protein S and protein C), resulting in increased risk of clotting, which often manifests as a purpuric rash - If the patient is immunocompromised, primary VZV infection could result in severe progressive disseminated disease (up to 20% mortality) - Vesicular eruptions may become haemorrhagic

Answer 31

- Admit if serious complications (e.g. pneumonia, encephalitis, dehydration) - In immunocompetent adolescents and adults, consider oral aciclovir 800 mg 5/day for 7 days if they present within 24 hours of the onset of the rash of if the chickenpox is severe - ADVICE - Encourage adequate fluid intake - Dress appropriately to avoid overheating or shivering - Wear smooth, cotton fabrics - Keep nails short - Explain that the most infectious period is 1-2 days before the rash appears - Infectivity continues until all the lesions are dry and crusted over (usually ~5 days after onset of the rash) - During this time, patients should AVOID contact with: - People who are immunocompromised - Pregnant women - Infants < 4 weeks - Children should be kept away from school until the vesicles have crusted over - Seek urgent medical advice if the condition deteriorates or they develop complications: - Bacterial superinfection - sudden high-grade pyrexia with erythema and tenderness around the original chickenpox lesions - Dehydration - e.g. reduced urine output, lethargy, cool peripheries - Immunocompromised children - Should be treated with IV aciclovir - Oral valaciclovir may be substituted later on - Prevention in immunocompromised patients - Human varicella zoster immunoglobulin for high-risk individuals with deficient T cell function following contact with chickenpox - NOTE: this protection is NOT absolute

Answer 32

- UNCOMMON in children - Caused by reactivation of latent VZV - Characteristically causes a vesicular eruption in the dermatomal distribution of the sensory nerves - Most commonly affects the thoracic region - Recurrent or multi-dermatomal shingles is strongly associated with underlying immunocompromise (e.g. HIV infection) - NOTE: in immunocompromised patients, reactivation of the infection can also disseminate and cause severe disease

Answer 33

- EBV causes infectious mononucleosis - It is also involved in the pathogenesis of: - Burkitt lymphoma - Lymphoproliferative disease - Nasopharyngeal carcinoma - EBV has a tropism for B lymphocytes and epithelial cells of the oropharynx - It is transmitted by oral contact - Most infections are subclinical

Answer 34

- Older children (and sometimes younger children) may develop a syndrome with: - Fever - Malaise - Tonsillitis/pharyngitis - Often severe enough to limit fluid and food intake - Lymphadenopathy - Prominent cervical lymph nodes, often with diffuse lymphadenopathy elsewhere - Other features - Petechiae of the soft palate - Splenomegaly (50%) - Hepatomegaly (10%) - Maculopapular rash - Jaundice

Answer 35

- Very clinical but can be supported by certain laboratory findings - Atypical lymphocytes (numerous large T cells) - Positive monospot test - Detects the presence of heterophile antibodies - NOTE: this test is often negative in young children with EBV - Seroconversion with production of THREE antibodies: - Viral capsid antigen antibodies (VCA) IgG and IgM - EB nuclear antigen (EBNA) antibodies - Symptoms of infectious mononucleosis may persist for 1-3 months but will ultimately resolve

Answer 36

- Paracetamol or ibuprofen to relieve pain and fever - Explain the expected course of the illness (2-3 week duration) - They don't need to avoid work or school but should do it depending on how they feel - Limit the spread of disease - Avoid collision/contact sport - Corticosteroids may be considered if the airway is severely compromised (RARE) - Seek medical help if: - Stridor or respiratory difficulty - Difficulty swallowing fluids or signs of dehydration - Systemically very unwell - Abdominal pain (e.g. due to splenic rupture) - WARNING: ampicillin and amoxicillin can cause a florid maculopapular rash in children infected with EBV so should be AVOIDED

Answer 37

- Usually transmitted via saliva, genital secretion or breastmilk - Causes mild or subclinical infection in normal paediatric and adult hosts - About 50% of adults show serological evidence of past infection - In the immuncompromised host and the developing foetus (transmitted from the mother), CMV is an important pathogen that can cause considerable morbidi

Answer 38

- Pharyngitis and lymphadenopathy are NOT as prominent as in EBV - Atypical lymphocytes on the blood film - They will be heterophile antibody negative

Answer 39

- Retinitis - Pneumonitis - Bone marrow failure - Encephalitis - Hepatitis - Oesophagitis - Enterocolitis

Answer 40

- Transplant recipients are closely monitored for evidence of CMV reactivation by blood PCR - This is why CMV-negative blood is used for transfusions - Antiviral prophylaxis may also be used

Answer 41

- CMV is self-limiting - If necessary, CMV can be treated with: - IV ganciclovir - Oral valganciclovir - Foscarnet - NOTE: these all have serious side-effects

Answer 42

- These two viruses are closely related and have similar presentations - HHV6 is more prevalent - MOST CHILDREN are infected by HHV6 o HHV7 by the age of 2 years, usually from the oral secretions of a family member - These viruses classically cause exanthema subitum (aka roseola infantum) - This is characterised by a high fever and malaise lasting a few days - This is followed by a generalised macular rash, which appears at the fever wanes - Many children will have the fever without the rash and many will have a subclinical infection - NOTE: this is often misdiagnosed as measles or rubella - NOTE: infants seen in the febrile stage may be given antibiotics, and when the exanthema subitum rash appears, they can be misdiagnosed with an allergy to antibiotic

Answer 43

- The condition will resolve over a few days/week - Paracetamol or ibuprofen for symptomatic relief - Advise to maintain adequate hydration - Explain risk of febrile seizures

Answer 44

- Causes erythema infectiosum (aka fifth disease) - It is commonly known as slapped-cheek syndrome - Outbreaks are most common during the spring - Transmitted via respiratory secretions or by vertical transmission from mother to foetus - It can also be transmitted via infected blood products - HPV-B19 infects the erythroblastoid red cell precursors in the bone marrow - It is mild and self-limiting - Advice on adequate rest and fluid intake - It is NOT necessary to stay away from school, but they may need to avoid contact with pregnant women

Answer 45

- Asymptomatic infection - About 65% of adults have antibodies against HPV-B19 - Erythema Infectiosum - MOST COMMON - It has a viraemic phase of fever, malaise, headache and myalgia - This is followed by a characteristic rash on the face (slapped-cheek), roughly a week later - This progresses into a maculopapular, 'lace-like' rash on the trunk and limbs - Complications are rare in children - In adults, it can cause arthralgia and arthritis - Aplastic Crisis - MOST SERIOUS - Occurs in children with chronic haemolytic anaemias (e.g. sickle cell disease, thalassemia) - Foetal Disease - Can lead to foetal hydrops and DEATH due to severe anaemia - Most infected foetuses will recover

Answer 46

- Paracetamol or ibuprofen - Adequate fluid intake - Secondary arthritis may be treated with ibuprofen

Answer 47

- There are many enteroviruses including - Coxsackie virus - Echoviruses - Polioviruse - Transmitted via the faecal-oral and respiratory droplet routes - Mainly occur in the summer and autumn - Over 90% of infections are ASYMPTOMATIC or cause non-specific febrile illness - Sometimes cause a blanching rash on the trunk and consists of fine petechiae - Some children may have loose stools or vomiting - The child is NOT usually systemically unwell

Answer 48

(Enterovirus) - Painful vesicular lesions on the hands, feet, mouth and tongue - Systemic features are generally mild - Disease subsides within days

Answer 49

(Enterovirus) - Vesicular and ulcerated lesions on the soft palate and uvula leads to anorexia, painful swallowing and fever - Severe cases may require IV fluids and analgesia

Answer 50

- In developed countries, enteroviruses are the MOST COMMON cause of viral meningitis - Most cases make a full recovery

Answer 51

(Enterovirus) - An acute illness with - fever - pleuritic chest pain - muscle tenderness - Recover within days

Answer 52

- RARE - Children may present with chest pain and/or heart failure associated with a febrile illness and evidence of myocarditis on ECG

Answer 53

- RARE - Occurs in the first few weeks of life - Results from transplacental/intrapartum infection of the infant - The symptoms are often SEVERE, mimicking bacterial sepsis - Affected infants may present with hypotension and multiorgan failure - Intensive care support is required - IMPORTANT: there are NO antiviral drugs that are effective against enteroviruses and the use of IVIG remains controversial

Answer 54

- Encephalitis - RARE - Initial symptoms are headache, lethargy and irritability - This proceeds to seizures and coma - Up to 15% mortality - Serious long-term sequelae include: seizures, deafness, hemiplegia, severe learning difficulties - Subacute Sclerosing Panencephalitis (SSPE) - RARE but devastating - Occurs, on average, 7 years after measles infection - Caused by a variant of the measles virus that persists in the CNS - Presents with loss of neurological function - This progresses, over several years, to dementia and death - Older children and adults tend to have MORE SEVERE disease - NOTE: in low-income countries, where malnutrition and vitamin A deficiency leads to impaired cell-mediated immunity, measles often follows a protracted course with severe complications

Answer 55

- Immediately notify the local Health Protection Team (HPT) - Self-limiting disease but it is likely to cause unpleasant symptoms such as rash, fever, cough and conjunctivitis - Rest and drink plenty - Paracetamol or ibuprofen can be used for symptomatic relief - Stay away from school for at least 4 days after the development of the rash - Seek urgent medical advice if they develop complications such as: - Shortness of breath - Uncontrolled fever - Convulsions or altered consciousness - Encourage vaccinations once the acute episode has subsided - Find out the immunisation status of close contacts - Children should be isolated in hospital - In immunocompromised patients, ribavirin may be of use - Vitamin A is given in low-income countries

Answer 56

- Spread by droplet infection to the respiratory tract where the virus replicates within epithelial cells - The virus gains access to the parotid glands before further dissemination to other tissues

Answer 57

- Incubation period: 15-24 days - The disease begins with a fever, malaise and parotitis - This may initially be unilateral, but it will eventually become bilateral after a few days - In up to 30% of cases, the infection will be subclinical - Parotitis is uncomfortable, and children may complain of earache or pain when eating/drinking - Examination of the parotid duct may show redness and swelling - The fever usually subsides after 3-4 days - Plasma amylase levels are usually elevated due to parotid inflammation - When associated with abdominal pain, consider pancreatic involvement - Infectivity lasts for up to 7 days after the onset of parotid swelling - The illness is usually mild and self-limiting - Hearing loss can rarely follow mumps, but it is usually unilateral and transient

Answer 58

- Notify the local Health Protection Unit (HPU) - Oral swab to confirm diagnosis - Self-limiting condition - Rest and take in adequate fluids - Paracetamol and ibuprofen for symptomatic relief - Stay away from school for 5 days after parotitis develops - Consider follow-up 1 week after the onset of parotitis - Seek help if they experience symptoms suggestive of meningitis or epididymo-orchitis - Find out the immunisation status of close contacts and tell them to watch out for symptoms of mumps

Answer 59

- Lymphocytes will be seen in the CSF in about 50% of cases - Typical meningeal signs are only seen in 10%

Answer 60

- MOST FEARED complication - Usually unilateral - Infertility is unusual after mumps orchitis

Answer 61

- Mild disease in childhood - Can cause severe damage to the foetus - Incubation period: 15-20 days - Spread by the respiratory route - May have a prodrome of low-grade fever or no symptoms at all - A maculopapular rash is often the first sign of infection - It tends to initially appear on the face and spread centrifugally to cover the whole body - Fades in 3-5 days - Lymphadenopathy, particularly suboccipital and postauricular nodes, is prominent - Complications are RARE in children (but include arthritis, encephalitis, thrombocytopaeina and myocarditis) - Diagnosis is confirmed by serology - There is NO effective antiviral treatment - Prevention is dependent on immunisation

Answer 62

- Contact the local Health Protection Unit (HPU) - Diagnosed using an oral fluid sample - Advise that it is a self-limiting disease - Rest and take in adequate fluids - Consider admitting if there is a serious complication such as haemorrhagic complications (caused by thrombocytopaenia) or encephalitis

Answer 63

- Bacterial infections e.g. typhoid, Bartonella henselae (cat scratch disease), Brucella - Deep abscesses e.g. intra-abdominal, retroperitoneal, pelvic - Infective endocarditis - Tuberculosis - Viral infections: HIV, CMV, EBV

Answer 64

- Systemic juvenile idiopathic arthritis (SJIA) - Systemic Lupus Erythematosus (SLE) - Vasculitis (Kawasaki disease) - IBD - Sarcoidosis - Malignancy e.g. leukaemia, lymphoma, neuroblastoma

Answer 65

- Systemic vasculitis - RARE, but important to recognise because aneurysms of coronary arteries are potentially devastating - It mainly affects children aged 6 months to 4 years - More common in Japanese children - Young infants tend to be more severely affected and are more likely to have incomplete symptoms (i.e. not the full list of cardinal features) - The diagnosis is clinical - Patients will have high inflammatory markers (CRP, ESR, WCC) with a platelet count that rises in the 2nd week of the illness - Coronary arteries are affected in about 1/3 of children within the first 6 weeks of the illness - This can lead to aneurysms which can be detected on echocardiography - Subsequence narrowing of the vessels due to scarring can lead to myocardial ischaemia and sudden death

Answer 66

- CRASH and Burn - Conjunctivitis - Rash - Adenopathy (usually cervical) - Strawberry tongue - Hand - swelling or erythema on the hands and feet - Burn - fever - This fever is usually difficult to control

Answer 67

- IVIG - High-dose aspirin (reduce thrombosis risk) - Other treatment options include corticosteroids, infliximab and plasma exchange - Children with giant coronary artery aneurysms may require long-term warfarin and close follow-up - Patients should, therefore, have a cardiovascular risk assessment - Children with persistent inflammation and fever may require corticosteroids, infliximab or ciclosporin

Answer 68

- Emergence of multi-drug resistance strains - Transmitted by the respiratory route - NOTE: latent TB is more likely to transform into active TB in infants and young children

Answer 69

- Diagnosis in children is particularly difficult - Clinical features are often non-specific - Prolonged fever - Malaise - Anorexia - Weight loss - Focal signs of infection (e.g. lymph node swelling) - NOTE: extra-pulmonary disease is relatively uncommon (e.g. TB lymphadenitis, osteoarticular TB, genitourinary TB, TB meningitis) - Sputum samples are unobtainable in children < 8 years old - Gastric washings on 3 consecutive mornings can be used to identify M. tuberculosis using Ziehl-Neelsen stains or auramine stains and mycobacterial cultures - This is obtained by passing an NG tube and washing out secretions in the stomach with saline - TB is difficult to culture but it should still be attempted because it is important to identify the antibiotic sensitivities - PCR-based methods are increasingly being used in parallel with mycobacterial cultures but these methods provide limited information about drug resistance - Tuberculin Skin Test (TST) - IGRAs - IMPORTANT: neither the IGRA or the TST can reliably distinguish between latent TB and active TB - NOTE: with advanced immunocompromise (e.g. HIV) both TST and IGRA can produce false negatives - BEWARE: do NOT attempt to diagnose TB based on CXR alone in HIV patients because lymphoid interstitial pneumonitis can have a similar appearance and occur in 20% of HIV-infected children - IMPORTANT: ALL individuals with TB should be tested for HIV and vice versa

Answer 70

- Injecting purified protein derivative intradermally into the forearm and observing 48-72 hours later and measuring the induration in millimetres - NOTE: PPD is a mixture of proteins, some of which are expressed by M. tuberculosis and the BCG, so a positive test result may be because of past BCG vaccination rather than latent TB - Originally, previous vaccination was taken into account when interpreting the TST result - NEW GUIDELINES: state that an induration of > 5 mm should be considered to be POSITIVE regardless of prior BCG vaccination

Answer 71

- Interferon Gamma Release Assay - New blood-based test - Assesses the response of T cells to in vitro stimulation with a small number of antigens that are expressed by M. tuberculosis but NOT BCG - Therefore, a positive result will indicate TB infection rather than BCG - HOWEVER, a negative result does NOT reliably rule out TB infection

Answer 72

- Admit if the patient has suspected active TB and is unwell - If hospital admission is not needed, urgent referral to specialist TB service to confirm diagnosis - It is a notifiable disease - Medical Management - Rifampicin + Isoniazid - 6 months - Pyrazinamide + Ethambutol - first 2 months - In adolescents, pyridoxine (vitamin B6) is given weekly to prevent peripheral neuropathy due to isoniazid - Dexamethasone in tuberculous meningitis to reduce the risk of long-term sequelae - IMPORTANT: asymptomatic children who are Mantoux or IGRA positive (i.e. latently infected) should also be treated as it will decrease the risk of reactivation later in life - Risk assessment for drug-resistant TB - A multidisciplinary approach should be taken including a key worker who should monitor the patient's adherence to treatment, clinical response and any adverse effects - Do contact tracing - TB Alert is a good website with information about TB

Answer 73

- BCG does reduce incidence of TB but its protective effect is incomplete - UK RECOMMENDATION: BCG is given at birth for high-risk groups - IMPORTANT: BCG should NOT be given to HIV-positive or other immunocompromised children - As most children are infected by a household contact, it is important to screen other family members - NICE GUIDELINES: children < 2 years who had close contact with a sputum smear positive pulmonary TB person should be started on prophylactic isoniazid - If TST and IGRA are negative at 6 weeks, isoniazid should be discontinued and BCG should be given

Answer 74

- There are several in the environment - Immunocompetent individuals rarely get affected by these organisms - May occasionally cause persistent lymphadenitis in young children - TREATMENT: complete lymph node excision or watchful waiting (in most cases, spontaneous resolution occurs) - NOTE: these organisms are transmitted by soil and water so NO contact tracing is needed - It can cause disseminated infection in immunocompromised individuals - Mycobacterium avium intracellulare infections are particularly common in patients with advanced HIV - These infections do NOT respond to conventional anti-TB treatment - Patients with cystic fibrosis are particularly susceptible to these infections

Answer 75

- Fever and a recent history of foreign travel - However, some tropical infections to consider are: - Malaria - Typhoid - Viral infections transmitted by mosquitoes (e.g. dengue fever, chikungunya, Zika virus) - Gastroenteritis and dysentery - Viral haemorrhagic fevers (e.g. Lassa, Marburg, Ebola, Crimean-Congo viruses

Answer 76

- Most affected children are in Sub-Saharan Africa - The main route of transmission in children is mother-to-child transmission which can occur: - Intrauterine (during pregnancy) - Intrapartum (at delivery) - Postpartum (through breastfeeding) - Uncommon routes of transmission to children include infected blood products, contaminated needles and sexual abuse

Answer 77

- Children > 18 months: detect antibodies against the virus - Children < 18 months who are born to infected mothers will have transplacental maternal IgG HIV antibodies, so a positive antibody test will confirm HIV exposure but not infection - The most sensitive test in this age group is HIV RNA PCR - ALL babies born to HIV-infected mothers should be tested, irrespective of whether they are symptomatic or not

Answer 78

- Some infants will progress rapidly to symptomatic disease and AIDS within the first year of life - Others are asymptomatic for months or years - Presentation depends on the degree of immunocompromise - Mild: lymphadenopathy or parotid enlargement - Moderate: recurrent bacterial infections, candidiasis, chronic diarrhoea, lymphocytic interstitial pneumonitis - SEVERE: Pneumocystic jirovecii pneumonia, severe growth faltering, encephalopathy, malignancy (RARE) - IMPORTANT: an unusual constellation of symptoms (especially infectious) should make you consider HIV

Answer 79

- Decision to start is based on a combination of clinical status, HIV viral load and CD4 count - IMPORTANT: infants should start ART shortly after diagnosis because they are at higher risk of disease progression - PCP prophylaxis with co-trimoxazole is given to infants who are HIV-infected, and for older patients with low CD4 counts - Other aspects of management: - Immunisation (except BCG) - MDT approach - Regular follow-up with particular attention to weight and developmental progress - Advise on risk reduction strategies - Reducing Vertical Transmission

Answer 80

- Mothers with a high viral load are more likely to transmit HIV to their infant - Avoidance of breastfeeding also reduces transmission - In high-income countries, perinatal transmission of HIV is < 1% due to: - Use of effective ART during pregnancy and intrapartum to achieve an undetectable maternal viral load at delivery - Post-exposure prophylaxis given to infant - Avoidance of breastfeeding - Active management of labour/delivery to avoid prolonged rupture of the membranes and unnecessary instrumentation - Pre-labour caesarean section if the mother's viral load is detectable close to the due date

Answer 81

- Caused by spirochaete Borrelia burgdorferi - Transmitted by hard ticks - History of tick bite is only elicited in about half of cases - Infections most commonly occur in the summer months in rural settings

Answer 82

- Incubation period: 4-20 days - Erythematous macule at the site of the tick bite enlarges to cause erythema migrans - Erythema migrans is described as a painless, red, expanding lesion with a bright red outer spreading edge - Early Features - Fever (fluctuate over several weeks and resolve) - Headache - Malaise - Myalgia - Arthralgia - Lymphadenopathy - NOTE: dissemination of infection early is RARE but can lead to cranial nerve palsies, meningitis, arthritis or carditis - Late Features (weeks or months after initial infection) - Neurological (meningoencephalitis, cranial and peripheral neuropathies) - Cardiac (myocarditis, heart block) - Joint (varies from brief migratory arthralgia to acute asymmetric monoarthritis and oligoarthritis of the large joints) - Occurs in 50% of patients - Recurrent attacks of arthritis are common - Chronic erosive joint disease can occur months/years after the initial attack in 10% of cases

Answer 83

- Based on clinical and epidemiological features and serology - Isolation of the organism is difficult - If Lyme disease is suspected without erythema migrans, offer an ELISA for Lyme disease - If the ELISA is positive, perform an immunoblot test - If immunoblot is positive, start treatment with oral antibiotics

Answer 84

- 1st line: DOXYCYCLINE - 2nd line: AMOXICILLIN - 3rd line: AZITHROMYCIN - IV ceftriaxone is needed for carditis or neurological disease - ADVICE - Explain that it is a bacterial infection treated with antibiotics but it can take some time (around a month) to get better - Some people can have a Jarish-Herxheimer reaction (antibiotics cause lysis of bacterial membranes leading to the release of toxins causing symptoms to worsen) - Lyme disease does NOT give lifelong immunity

Answer 85

- Diphtheria - Tetanus - Pertussis - Polio - Hib - Hepatitis B

Answer 86

- 2 months - 3 months - 4 months

Answer 87

- 2 months - 4 months - 12 months

Answer 88

- 2 months - 4 months - 12 months

Answer 89

- 2 months | - 3 months

Answer 90

- Booster Hib and MenC is given at: - 1 year - MMR is given at : - 1 year - 3 years 4 months - HPV is given to girls at: - 12-13 years - Meningococcal ACWY conjugate vaccine is given at : - 14 years

Answer 91

- Swelling and discomfort at the injection site with mild fever and malaise - Anaphylaxis can occur but is very rare - Live vaccines should NOT be given to immunocompromised children (except HIV-positive children on ART) - Vaccination should be postponed if the child has an acute illnes

Answer 92

- Primary (RARE): genetically determined defect of the immune system - Secondary: caused by another disease or treatment such as malignancy, chemotherapy, malnutrition, HIV, immunosuppressive therapy, splenectomy or nephrotic syndrome

Answer 93

- Most are X-linked recessive or autosomal recessive - May be a family history of consanguinity or unexplained death - Primary immunodeficiency should be considered in children presenting with Severe, Prolonged, Unusual or Recurrent infections

Answer 94

- Recurrent (proven) bacterial infections - Severe infections (e.g. meningitis, osteomyelitis, pneumonia) - Infections that present atypically, are unusually severe or chronic or fail to respond to regular treatment - Infections caused by an unexpected or opportunistic pathogen or a pathogen the child has been immunised against - Severe or long-lasting warts, generalised molluscum contagiosum - Extensive candidiasis - Complications following live vaccinations (e.g. disseminated BCG) - Abscesses of internal organs; recurrent skin abscesses - Prolonged or recurrent diarrhoea (often combined with faltering growth)

Answer 95

- Antimicrobial prophylaxis - For T-cell and neutrophil defects: - Co-trimoxazole to prevent PCP - Itraconazole or fluconazole to prevent other fungal infections - For B-cell defects: - Antibiotic prophylaxis (e.g. azithromycin) to prevent recurrent bacterial infection - Antibiotic treatment - Prompt treatment of infections - Longer courses - Low threshold for IV therapy - Screening for end-organ disease - E.g. CT scan in children with antibody deficiency to detect bronchiectasis - Immunoglobulin replacement therapy - For children with antibody deficiency - Bone marrow transplantation - E.g. for SCID, chronic granulomatous disease - Gene therapy

Answer 96

- Pathological immune response occurs against a specific food protein - Usually IgE mediated - A non-immunological hypersensitivity reaction to a specific food is called food intolerance

Answer 97

- This is usually due to cross-reactivity between proteins present in fresh fruits/vegetables/nuts and those present in pollens - E.g. a child who can eat apples may develop an allergy to apples when they are older if they develop an allergy to birch tree pollen, which has a very similar protein - This is called pollen food allergy syndrome - It can cause mild allergic reactions (e.g. itchy mouth)

Answer 98

- Infants: most common causes are milk, eggs and peanut | - Older Children: peanut, tree nut, fish, shellfish

Answer 99

- History of allergic symptoms varying from urticaria to facial swelling to anaphylaxis - Allergy occurs 10-15 mins after ingestion of a food - Non-IgE mediated food allergy usually presents with diarrhoea, vomiting, abdominal pain and sometimes faltering growth - Colic or eczema may also be present - It may present with blood in the stools due to proctitis in the first few weeks of life or severe repetitive vomiting which can result in shock (food protein-induced enterocolitis syndrome)

Answer 100

- typically occurs hours after ingestion and usually involves the GI tract

Answer 101

- Clinical history - For IgE mediated food allergy, skin-prick tests are the most useful - Patch testing - identify allergens that cause reactions through delayed contact hypersensitivity where T cells play a major role - The allergenic extract is held against the skin for 48 hours - Measurement of specific IgE antibodies in the blood is also useful - The greater the result, in both tests, the more likely the child is to be allergic - Non-IgE mediated food allergies more reliant on clinical history and examination - Endoscopy and intestinal biopsy are sometimes performed - The diagnosis of non-IgE mediated food allergy is supported by the presence of eosinophilic infiltrates - IMPORTANT: for both IgE and non-IgE mediated food allergies, the GOLD STANDARD investigation in cases of doubt is EXCLUSION of the relevant food under the dietician's supervision - This is followed by a double-blind placebo-controlled food challenge - This involves the child being subject to increasing amounts of the food or placebo - It should be performed at hospital with full resuscitation facilities available

Answer 102

- Avoidance of the relevant food - Educating the child and family about how to manage an allergic attack (allergy action plan) - Provide written self-management plans and training - Mild reactions (no cardiorespiratory symptoms) are treated with non-sedating antihistamines - Severe reactions (with cardiovascular, laryngeal or bronchial involvement) require IM adrenaline (may be given by autoinjector (EpiPen)) - Food allergy to cow's milk and egg often resolves in early childhood, so gradual reintroduction may be possible - Food allergy to nuts and seafood usually persist through to adulthood

Answer 103

- Abnormal reaction by the body's immune system to a protein found in cows' milk - Classification - IgE-mediated = immediate reaction - Non-IgE Mediated = delayed reaction

Answer 104

- GI symptoms: vomiting, abdominal pain, blood in stools, diarrhoea - Skin symptoms: hives, eczema - Babies: wheezing, irritability, facial swelling, poor growth

Answer 105

- History and examination | - Consider referral to secondary care for a skin prick and/or specific IgE antibody blood test

Answer 106

- Eliminate cow's milk for at least 6 months or until the child is 9-12 months - Breastfed Babies: advise mother to exclude cows' milk protein from her diet. Consider prescribing daily supplement of 1000 mg of calcium and 10 mcg of vitamin D - Formula-fed Babies: advise replacement of cows' milk-based formula with hypoallergenic infant formula (e.g. extensively hydrolysed formula or amino acid formula) - Weaned infants/older children: exclude cows' milk protein from their diet - Offer nutritional counselling with a paediatric dietician - Regularly monitor growth - Re-evaluate the child to assess for tolerance to cows' milk protein (every 6-12 months) - this involves re-introducing cows' milk protein into the diet - If tolerance is established, greater exposure of less processed milk is advised following a 'Milk Ladder' - SUPPORT: British Dietetic Association (BDA) has produced a useful fact sheet

Answer 107

- This can be atopic or non-atopic - It is classified based on pattern and severity of symptoms - Pattern: intermittent or persistent - Severity: mild, moderate or severe - It can also be classified as seasonal or perennial - It can also present with a cough due to post-nasal drip or chronically blocked nose leading to sleep disturbance

Answer 108

- Assess for symptoms of asthma - Try to identify the most likely causative allergen - Look for signs of chronic nasal congestion (e.g. mouth breathing, cough, halitosis) - Examine the nose for nasal polyps, deviated nasal septum, mucosal swelling or depressed or widened nasal bridge - For those who want 'as required' treatment for occasional symptoms - If aged 2-5 or preference for oral treatment = oral antihistamine (e.g. cetirizine) - All others = intranasal azelastine (antihistamine) - For those who want preventative treatment for frequent symptoms - Avoid the causative agent - If main issue is nasal blockage or nasal polyps = intranasal corticosteroid (e.g. beclometasone) - If main issue is sneezing or nasal discharge = oral antihistamine or intranasal corticosteroid - For people requiring rapid relief whilst waiting for preventive treatment to take effect - Intranasal corticosteroids for up to 7 days - Consider adding oral antihistamine - If symptoms are severe and/or impairing quality of life, prescribe a 5-10 day course of prednisolone

Answer 109

- Viral infection (rash lasts for days) | - Allergen exposure (rash lasts for hours)

Answer 110

- Urticaria can involve deeper tissues to produce swelling (angioedema), especially of the lips and soft tissues around the eyes

Answer 111

- Hives or redness and results from local vasodilation and increased permeability of capillaries and venules - This is dependent on activation of skin mast cells, leading to the release of various mediators including histamines - Itchy - When allergy has caused urticaria, there is a risk of anaphylaxis - Chronic urticaria (> 6 weeks) is usually non-allergic

Answer 112

- Identify and manage underlying triggers - Symptom diaries can be useful - Consider assessing severity using a tool such as Urticaria Activity Score (UAS7) - Advise that urticaria is likely to be self-limiting - If symptomatic: - Offer non-sedating antihistamine (e.g. cetirizine) for up to 6 weeks - If severe, offer oral corticosteroid - If symptoms improve, consider giving treatment to take as required (if there were to be future episodes) or daily antihistamines for 3-6 months if symptoms are likely to be persistent - Consider referral to the dermatologists - In refractory cases, leukotriene receptor antagonists or anti-IgE antibody (omalizumab) may be used

Answer 113

- Antibiotics allergy is common in children - A lot of children who have been labelled as 'drug allergic' are NOT truly allergic - This is because viral illnesses may cause a delayed rash, before which antibiotics are often erroneously given to the child

Answer 114

- Arises mainly from bee and wasp stings - Severity may be: - Mild: local swelling - Moderate: generalised urticaria - Severe: systemic symptoms with wheeze or shock - Those who have suffered from a severe reaction should be offered an EpiPen and allergen immunotherapy

Answer 115

- Acute inflammation of the vermiform appendix

Answer 116

- Obstruction of the appendiceal lumen, causing a cycle of progressive inflammation and bacterial outgrowth

Answer 117

- Most common cause of an acute abdomen in children - Incidence: 4/100 - Any age, most common >5 years of age, uncommon <2 years

Answer 118

- Large variation in clinical picture - Periumbilical colicky pain then localises to the right iliac fossa. Constant with peritoneal inflammation and increased with movement - Anorexia (vague abdominal pain and won't eat their favourite food) - Vomiting (young children) - Constipation or diarrhoea (less common) - Low grade pyrexia

Answer 119

- General: Tachycardia, pyrexia, reluctance to move - Abdominal examination: Percussion tenderness signifies inflammation of the peritoneum . Guarding may be present in RIF (McBurney's point). Rovsing's sign (LIF palpation causing RIF pain). There may also be pain on expansion and recession of the abdomen. Cough may exacerbate pain. Peritoneal irritation signs may be absent with a retrocaecal appendicitis - Rectal examination: Should be performed by the most senor doctor only when diagnosis is in doubt. There is marked tenderness against rectal wall, especially with a retrocaecal appendix

Answer 120

- General: Appendicitis is a clinical diagnosis: investigations may aid diagnosis in difficult cases. - Bloods: Increased WCC (normal WCC doesn't exclude appendicitis), increased CRP, U&Es (especially if vomiting), clotting (raised neutrophil count is the most sensitive serological investigation for appendicitis) - Urine: MC&S to exclude UTI, leucocytes may be present with an inflamed appendix against bladder wall (nitrite -ve) - Radiology: Plain AXR not indicated; if performed, may show dilated loops of bowel and a fluid level in the RIF. USS may show the inflamed appendix as a non-compressible tubular structure, presence of free fluid or appendiceal mass

Answer 121

- Surgical: once diagnosed is confirmed clinically. May be performed via the traditional open approach (Lanz incision) or laparoscopically. Washout essential with complicated appendicitis - Conservative: With a confirmed appendiceal abscess that responds to intravenous antibiotics. If this management fails then surgical intervention (+/- drain insertion) is indicated. If conservative management succeeds then the patient is offered an interval appendicectomy

Answer 122

- Perforation: <3 years old = 80-100%; >10 years old = 10-20%. Complicated appendicitis (perforated/presence of pus); wound infection/intra-abdominal abscess formation. - Decreased fertility in girls after complicated appendicitis (ovarian/fallopian tube involvement), small bowel obstruction, adhesions

Answer 123

- Usually excellent

Answer 124

- Chronic inflammatory airways disease characterised by - variable reversible airway obstruction - airway hyper-responsiveness - bronchial inflammation

Answer 125

- Result from small airways obstructing and becoming narrow due to inflammation and aberrant immune responses to viral infection - MOST wheezy preschool children have viral episodic wheeze - Viral episodic wheezing usually resolves after about 5 years (probably due to an increase in airway size)

Answer 126

- Genetic factors: Positive family history of asthma or atopy. - Environmental triggers: Passive or active smoking, URTIs, exercise, cold weather, inhalant allergies (house dust mite/pollens/moulds/pets) and food allergens

Answer 127

- RISK FACTORS: maternal smoking during and/or after pregnancy and prematurity

Answer 128

- Prevalence: 10–15%. Age: 80% of asthmatic children are symptomatic by the age of 5. M: F, 2:1; equalises in adulthood. - Distribution: Viral-associated wheeze/recurrent wheezy bronchitis " in urban areas and in children of low socio-economic status families.

Answer 129

- Age-related symptoms - <1 year: Persistent or recurrent nocturnal cough, wheezing with URTIs. - 2–3 years: Nocturnal cough, wheezing during exercise with URTIs. - <5 years: Non-productive cough may be the only symptom, often worse at night and in the morning. - Assess severity: Frequency of attacks (mild: <1 attack in 2 months; moderate: >1 attack in 2 months; severe: persistent symptoms, decreased exercise tolerance), effect on school attendance, hospital attendances and admissions to PICU.

Answer 130

- Respiratory: End-expiratory wheeze, recession, use of accessory muscles, tachypnoea, hyper-resonant percussion note, diminished air entry, hyperexpansion, Harrison sulcus (anterolateral depression of thorax at insertion of diaphragm). - Peak flow: Peak flow: Useful in >5 years of age; use as baseline (predicted best) and as determinant for efficacy of treatment

Answer 131

- Too breathless to speak - Tachycardia - > 120 bpm in 2-5 years - > 130 bpm in <2 years - Tachypnoea - > 30 breaths in 2-5 years - > 50 breaths in <2 years - Peak flow: <50% predicted > 5 years

Answer 132

- Silent chest - Cyanosis - Poor respiratory effort - Hypotension - Exhaustion - Confusion - Coma - Peak Flow: <33% predicted in >5 years

Answer 133

- CXR: In acute severe cases to exclude pneumothorax or first presentation to exclude congenital anomaly. - Lung function (spirometry): Can be performed in >5 years. Obstructive airways disease: FEV1 <80%, FVC normal or reduced, FEV1/FVC <70%. Assess reversibility after 400 mg salbutamol inhalation.

Answer 134

- High-flow oxygen via reservoir bag. - Salbutamol and ipratropium bromide via volumatic spacer or nebulised. - Oral prednisolone 20 mg (2–5 years), 30–40 mg (>5 years) or IV hydrocortisone if unable to retain oral medication. - Commence IV salbutamol (bolus then infusion) or aminophylline infusion. - Magnesium sulphate (40 mg/kg) IV. - If not responding (<92% O2 saturations) or any life-threatening features present, discuss with PICU for ventilatory support. - Discharge criteria: Patients can be discharged when stable on 3–4-hourly inhaled bronchodilators. Peak flow 75% of predicted best, and O2 saturations >94%. - Education: On adherence to medication, recognition of acute attacks, emergency protocol, maintaining normal activities

Answer 135

1. Avoid obvious precipitants, e.g. passive smoking, allergen avoidance. 2. Ensure good inhaler technique þ / volumatic spacer. 3. Check compliance. 4. Review treatment every 3–6 months. 5. ‘Rescue’ prednisolone in acute deterioration. 6. If obese advise weight reduction.

Answer 136

1. Symptomatic/use b2-agonist inhalers 3 times/week. 2. Waking one night/week. 3. Frequent exacerbations

Answer 137

Step 1, mild intermittent asthma: Short-acting b2-agonist inhalers (e.g. salbutamol) as necessary. Step 2, regular preventer control: Add low-dose inhaled steroid (200–400 mg/day budesonide equivalent) or leukotriene receptor antagonist if steroid cannot be used. Step 3, add-on therapy: Trial of leukotriene receptor antagonist. Step 4, persistent poor control: Refer to respiratory paediatrician.

Answer 138

- STEP 1: offer a SABA (e.g. salbutamol) as reliever therapy - STEP 2: consider an 8-week trial of paediatric moderate dose ICS in children with: - Symptoms at presentation that clearly indicate the need for maintenance therapy (e.g. asthma-related symptoms > 3 times per week) - Suspected asthma that is uncontrolled by SABA alone - STEP 3: Consider an LTRA in addition to the ICS and review in 4-8 weeks - STEP 4: Consider stopping the LTRA and starting a LABA - STEP 5: Consider changing the ICS and LABA maintenance therapy to a MART regimen, with paediatric low maintenance ICS dose - STEP 6: Consider increasing ICS to a paediatric moderate maintenance dose - STEP 7: refer to specialist care (they may consider using paediatric high dose ICS or a trial of an addition drug (e.g. theophylline))

Answer 139

- Decreased linear growth rate due to poorly controlled asthma more usual than from overprescription of inhaled steroids, chest wall deformity, recurrent infections, status asthmaticus can be fatal. - One-third of deaths occur under the age of 5 years

Answer 140

- Asthma often remits during puberty and many children are symptom free as adults, especially those who have mild asthma and are asymptomatic between attacks, or who develop asthma at >6 years. - Rates of admission and mortality in asthma have decreased since the early 1990s.

Answer 141

- A developmental condition in which the child experiences a brief episode of apnoea.

Answer 142

- Pallid (or white) breath-holding attacks: Abnormally sensitive response to carotid sinus or ocular compression with the production of temporary asystole or marked bradycardia. - Cyanotic (or blue) breath-holding attacks: Mechanism unclear; however, includes centrally mediated reduced respiratory effort and altered lung mechanics, which may inappropriately stimulate pulmonary reflexes, thus resulting in apnoea and hypoxia.

Answer 143

- Occurs in 1–2% of children between the ages of 6 months and 5 years, 75% of which occur between 6 and 18 months. M = F. - Breath-holding spells usually occur from 1–2/day to 1–2/month. 60% have cyanotic type, 20% pallid type and 20% a combination.

Answer 144

Pallid breath-holding attack - Triggered by fear or a painful stimulus such as a knock to the head or falling > - Child stops breathing and rapidly loses consciousness > - Child becomes pale and hypotonic > - May experience a tonic seizure as a result of cerebral underperfusion (reflex anoxic seizure) Cyanotic breath-holding attack - Triggered by anger, frustration, upsetting event or scolding > - Child cries and subsequently holds breath in expiration > - Rapid onset of cyanosis that may progress to loss of consciousness > - Brief tonic-clonic jerks, opisthotonos (rigidity and arching of the back, with head thrown backwards) > - Bradycardia may follow Attacks last less than a minute. There is usually full resumption of normal activity within minutes. Some children may remain lethargic and drowsy for some time after an attack.

Answer 145

- Neurological examination to exclude focal signs suggestive of an underlying structural abnormality if unusual features in history.

Answer 146

- Not usually required. - EEG: Generalised slow waves with flattening during the attack (a pattern characteristic of cerebral hypoxia). Interictally the EEG is normal. - ECG: If cardiac arrhythmia is suspected.

Answer 147

- Parental education: Reassurance and emphasis on consistency and not reinforcing the child's behaviour pattern after the attack. - Child should lie flat during attack for cerebral perfusion. - Atropine sulphate may be considered in refractory pallid attacks to block the vagus nerve.

Answer 148

- No immediate complications except if the child collapses in an unsafe environment

Answer 149

- Stop having the attacks after the age of 5 or 6 years. - Children who have pallid attacks have an " incidence of syncope in adulthood. - There is no increased incidence of epilepsy in either type.

Answer 150

- Respiratory condition characterised by coryza followed by a 'bronchiolitic' dry, wheezy cough, breathlessness, poor feeding, hyperinflation of the chest and expiratory wheeze in infants

Answer 151

- RSV (75%) | - There may be multiple causative agents (rhinovirus, parainfluenza, influenza or adenovirus).

Answer 152

- Most common LRTI in infants, especially 3–6 months. Winter epidemics: <=3% infants are admitted to hospital.

Answer 153

- Cough - Breathlessness - Wheeze. - In more severe cases infants may become too breathless to feed, have apnoeic spells or become lethargic

Answer 154

- General: Mild pyrexia, tachycardia, irritability. - Respiratory distress: Tachypnoea, subcostal/intercostal recession, nasal flaring, grunting, widespread expiratory wheeze +/- fine crepitations. - Severe disease: Cyanosis, lethargy.

Answer 155

- Bloods: Not indicated in mild disease. May find increased WCC, decreased Na (2˚ to SIADH), capillary gas (decreased pO2 and increased pCO2 if child is becoming exhausted) - CXR: Not indicated in mild disease; will show hyperinflation due to small airways obstruction and air trapping. Collapse (classically of the right upper lobe) and/or consolidation in secondary bacterial infection may also be seen. - Serology: RSV may be identified by immunofluorescent staining of nasopharyngeal aspirations (NPA) using specific viral antisera.

Answer 156

- Humidified oxygen supplementation if saturation is persistently < 92% - Consider CPAP if impending respiratory failure - Consider upper airway suction if there is evidence of increased airway secretions (this should definitely be performed if they are presenting with apnoea) - Give fluids by nasogastric or orogastric tube if they cannot take enough fluid by mouth - Supportive - IMPORTANT: RSV is highly infectious, so infection control measures are needed to prevent cross-infection - Most infants will recover within 2 weeks - RARELY, the illness may cause permanent damage to the airways (bronchiolitis obliterans)

Answer 157

- Humidified oxygen supplementation if saturation is persistently < 92% Consider CPAP if impending respiratory failure Consider upper airway suction if there is evidence of increased airway secretions (this should definitely be performed if they are presenting with apnoea) Give fluids by nasogastric or orogastric tube if they cannot take enough fluid by mouth Supportive IMPORTANT: RSV is highly infectious, so infection control measures are needed to prevent cross-infection Most infants will recover within 2 weeks RARELY, the illness may cause permanent damage to the airways (bronchiolitis obliterans)

Answer 158

- Mortality 0.2%, intensive care unit admission 2.7% and need for ventilatory assistance 1.5%. - Cardiac failure may occur, predominantly in infants with underlying CHD.

Answer 159

- Difficulty breathing, wheeze and poor feeding: 6–7 days, cough: 12 days. - Diarrhoea may complicate the recovery phase. - Persistent cough and recurrent viral-induced wheeze recur in 20% of infants (up to 60% of infants hospitalised). - Recurrent wheeze is more common in the first 5 years after RSV bronchiolitis, but there is conflicting evidence as to whether RSV bronchiolitis predisposes to asthma.

Answer 160

- Results when the heart can no longer meet the metabolic demands of the body.

Answer 161

- Refers to increased venous congestion in the pulmonary (left heart failure) or systemic (right heart failure) veins. - This occurs when the compensatory mechanisms used to improve cardiac output are no longer adequate, and heart failure becomes uncompensated

Answer 162

- Overcirculation: Normal forward blood flow is disrupted and the heart becomes an inefficient pump. 1. Congenital heart disease (CHD): hypoplastic left heart (HLH), severe aortic stenosis, interrupted aortic arch, coarctation of the aorta (COA), patent ductus arteriosus (PDA), total anomalous pulmonary venous drainage (TAPVD), large VSD, transposition of the great arteries (TGA), truncus arteriosus. 2. Postoperative repair of CHD. 3. Arteriovenous malformation, e.g. hepatic. 4. Severe anaemia, e.g. hydrops fetalis. - Pump failure: Heart muscle is damaged and no longer contracts normally 1. Viral myocarditis/cardiomyopathy 2. Metabolic cardiomyopathy (e.g. Pompe disease) 3. Arrhythmias: SVT, VT or congenital heart block (CHB) 4. Ischaemia (post Kawasaki disease) 5. Duchenne muscular dystrophy 6. Medications e.g. chemotherapy

Answer 163

- Rare in children; more common in infants with CHD.

Answer 164

- Infants: Breathlessness, wheeze (cardiac asthma), grunting, feeding difficulties, sweating, failure to thrive, recurrent chest infections. - Older children: Fatigue, exercise intolerance, dizziness or syncope.

Answer 165

- General: Tachycardia (not in CHB); absence of a heart murmur does not exclude heart disease (TGA, HLH, COA). - Left CHF: Tachypnoea, respiratory distress (recession), gallop rhythm, displaced apex. - Right CHF: Hepatosplenomegaly +/- oedema or ascites. Jugular venous distension is not a reliable indicator in infants and young children. - Uncompensated CHF: Hypotension, cool peripheries, prolonged capillary refill time, thready pulse, decreased urine output, signs of renal and hepatic failure in severe cases

Answer 166

- Bloods: Acid/base balance, electrolytes and osmolality (likely SIADH), liver and renal function. - CXR: Cardiomegaly, increased pulmonary vascular markings and fluid collection in the horizontal fissure/pleural effusions may be detected - ECG: Rate, rhythm, atrial/ventricular hypertrophy or hypoplasia. Evidence of myocarditis/cardiac ischaemia or ventricular strain. - ECHO: Diagnostic for CHD. - Cardiac catheterisation: Measures intracardiac pressures and shunts.

Answer 167

- Aims - Reduce preload - Using diuretics (e.g. furosemide) or, more rarely, venous dilators (e.g. nitroglycerin) - Enhance cardiac contractility - Using IV agents (e.g. dopamine) - Other options: digoxin, dobutamine, adrenaline, milrinone - Reduce afterload - Oral ACE inhibitors - IV agents (e.g. hydralazine, nitroprusside, alprostadil) - Improving oxygen delivery - Beta-blockers (e.g. carvedilol) - Enhancing nutrition - If there is heart failure due to a cardiac malformation - If cyanotic --> Prostaglandin infusion - This maintains a PDA in duct-dependent cyanotic heart disease and buys time before surgical correction can be performed - Echocardiography to identify the underlying structural defect is necessary

Answer 168

- Arrhythmias - SVT - VT - CHB - cardiogenic shock

Answer 169

- Poor in children in the absence of correctable CHD

Answer 170

- Non-progressive disorder of movement and posture.

Answer 171

- Antenatal (80%): Cerebral dysgenesis/malformation, congenital infections (rubella, toxoplasmosis, CMV). - Perinatal (10%): Hypoxic ischaemic encephalopathy, birth trauma. - Postnatal (10%): Meningitis, encephalitis, extradural haemorrhage, IVH, head injury, NAI, hyperbilirubinaemia (kernicterus), prolonged hypoglycaemia.

Answer 172

- 2/1000 live births. Usually presents in infancy

Answer 173

- General: Delayed milestones, poor feeding, abnormalities of tone, posture, gait, difficulties with language, impaired social skills Clinical Types - Spastic (70%): Affected limbs show increased tone (clasp-knife), brisk reflexes, extensor plantar responses: - Hemiplegia: unilateral, arm > leg, fisting and early hand preference <1 year, characteristic posture of abduction of shoulder, flexion at elbow and wrist, pronation of forearm, and extension of fingers - Diplegia: legs > arms, hypertonicity of hip adductors ! leg ‘scissoring’ - Quadriplegia: all 4 limbs affected – arms > legs, poor head control, paucity of movement. Abnormal primitive reflexes and fisting in the first few months. - Dyskinetic (10%): Normal progress until 6–9 months, followed by progressive dystonia of lower limbs, trunk, and mouth exaggerated by involuntary movements; athetoid (writhing) and choreographic movements (jerking). - Ataxic (10%): Hypotonia, ataxia of trunk and limbs, postural imbalance, intention tremor. - Mixed (10%).

Answer 174

- Assessment of hearing and vision. | - EEG if seizure prone

Answer 175

- Education: mainstream school + extra support (liaison with (SENCO)) or special needs school - Physiotherapy: early to maintain full ROM, function, normal development and prevent contractures. - Occupational therapy: splints, crutches, walking frames, wheelchairs. - SALT: including control of drooling (oral training). - Feeding: may be unable to suck, chew or swallow, therefore requiring gastrostomy feeds. - Orthopaedic: surgery to correct deformity and improve function. - Neurosurgical intervention: considered to reduce muscle spasticity or for disabling dystonic movements. - Medical: baclofen or botulinum toxin injections to relieve spasticity. Genetic counselling

Answer 176

- Aspiration pneumonia - Failure to thrive - Scoliosis - Dislocated hips.

Answer 177

- Spastic hemiplegia: Delayed but eventually normal gait. - Spastic diplegia: Characteristic gait (knees flexed, toe walking, and adducted hips). - Spastic quadriplegia: Poor prognosis related to feeding disability and immobility. Sufferers are often totally dependent and life expectancy is significantly reduced. Usually die from chest infections 2 to muscular weakness and poor chest dynamics 2 to kyphoscoliosis. - Dyskinetic: Usually unable to walk independently, quality of life can often be poor. - Ataxic: Most children walk (though often delayed) with the aid of crutches