Infectious Disease Flashcards

1
Q

What is the leading cause of acquired heart disease in children under 5 years of age in the US and other developed countries?

A

Kawasaki disease

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2
Q

What is Kawasaki disease and it’s aetiology?

A
Kawasaki disease (KD) is an acute, febrile, self-limiting, systemic vasculitis of unknown origin that almost exclusively affects young children. 
In an immunogenetically pre-disposed host, one or more infectious agents may play a role in triggering the clinical manifestations of the disease
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3
Q

How does Kawasaki disease present?

A

Acute febrile illness with vasculitis lasting 5 or more days.

5 cardinal areas; rash, mucosa, conjunctiva, extremities, lymph nodes.

Typical signs include fever, polymorphic rash – the rash of Kawasaki disease may be morbilliform (measles-like), maculopapular (red patches and bumps), erythematous (red skin) or target-like. Usually starts in perineum - nappy area.

injected eyes - red, conjunctivitis

Swelling and erythema of the hands and feet occur in the acute stage, followed by desquamation in the second week.

  • Swollen, red palms and soles (erythematous and oedematous), may affect mobility/ability to use hands – progresses to desquamation/peeling ~2 weeks ± Beau lines (transverse grooves across nails)
  • Erythema of oral/pharyngeal mucosa, strawberry tongue, cracked lips

Unilateral non-purulent cervical lymphadenopathy is present in about 40% of cases.

Coronary aneurysms develop in 20% to 25% of untreated patients.

Cracked lips

Other possibilities:
± pericarditis, CHF, joint pains, neuro (headaches, facial palsy), GI (abdo pain, D&V, obstructive jaundice), sterile pyuria, other derm

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4
Q

How do we ivx Kawasaki and what are the findings?

A

FBC: in acute phase – mild/mod normochromic anaemia, ↑WCC, in subacute phase (2nd week) - ↑plts
↑ESR, ↑CRP, ↑ferritin, ↑α1AT

Echo: evaluate for CAs (do a baseline at start, repeat week 2/3 of illness and at 2 months follow up)

Can consider: LFTs, urinalysis, CXR, ECG
Diagnosis is made on clinical findings

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5
Q

What are risk factors for Kawasaki?

A

Asian ancestry
age 3 months to 4 years
male sex

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6
Q

How do we manage acute Kawasaki? Prognosis?

A

Admit to hospital

presentation ≤10 days from onset; or presentation >10 days from onset with risk factors for complications

1st line: intravenous immunoglobulin (IVIG) - don’t give after 10 days
plus: high-dose aspirin

2nd line: corticosteroid
plus: high-dose aspirin

2nd line: infliximab - TNFa blocker
plus: high-dose aspirin

3rd line: other immunomodulatory drug or plasma exchange
plus: high-dose aspirin

Not acute:
presentation >10 days from onset without risk factors for complications
1st line: low-dose aspirin
- (as unlikely to now develop CAs)

After initial episode:
Stop aspirin
Unless there is aneurysm- in which case: start anti platelets eg clopidogrel

Follow up with echo essential to check for CAs at 2 months

Prognosis ; 2nd infection is rare

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7
Q

What is danger of aspirin in kids?

A

aspirin rarely used in children due to risk
of Reye syndrome (rapidly progressing
encephalopathy, liver failure, coma)

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8
Q

When is cardiovascular disease in Kawasaki likely to manifest? How do we monitor this?

A

From 3 weeks - 2 months post infection

Hence need for echo to monitor heart

May hear gallop rhythm / murmur

It’s not just the aneurysm that is the issue there can be pericarditis, myocarditis and sudden death

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9
Q

Measles, mumps, rubella are examples of?

A

Notifiable diseases

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10
Q

What is the aetiology and clinical presentation of mumps?

A

Mumps virus - RNA Paramyxovirus, transmission by resp droplets

Systemic infection - not just resp

Clinically:
• Headache, fever,
Painful Parotid swelling
• +/- pancreatitis, neuritis, arthritis, mastitis, nephritis, thyroiditis, pericarditis

Meninges involvement, gonads, pancreas ( causing mumps orchitis, encephalitis, aseptic meningitis and deafness)

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11
Q

Risk factors for mumps?

A
unvaccinated status
international traveller
immunosuppression
healthcare worker
close-contact living (college students, prisoners, military)
vaccine failure
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12
Q

How do we ivx mumps? Treatment? Prognosis?

A

salivary mumps IgM
Can do serology- Elisa
FBC - leukocytes is

Believe it’s clinical
Can ivx for complications eg serum Amylase for pancreatitis

Treatment:
No cure, supportive Tx with analgesia, fluids, infection should pass 1-2 weeks
• Prognosis – mumps is rarely fatal, but complications include infertility (oophoritis, orchitis), aseptic meningitis, deafness

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13
Q

Describe the measles virus? what is the mode of spread?

A

Is of the Paramyxoviridae family, genus morbilivirus
-> name: rubeola virus

spread through resp secretions
• One of the most highly communicable diseases
• Spending more than 15 mins in direct contact is enough to transmit
• 7-18 day incubation, period of infectivity from 4 days before and 4 days after rash

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14
Q

How does measles present?

A

High fever, coryxa, conjunctivitis

Koplik spots, small red spots with white centres clustered inside mouth/buccal mucosa - “grains of salt on a reddish background” :

  • are prodromic of measles and manifest 2-3 days before the measles rash itself.

• maculopapular Rash - craniocaudal spread (appears around hairline first) then on face/neck, spreads to hands and feet

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15
Q

How do we ivx measles?

A

measles-specific IgM and IgG serology (ELISA)

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16
Q

How do we manage measles? Complications?

A

Rest and supportive treatment – fluids (maintaining good hydration), antipyretics

Vitamin A supplements - to boost antibody response

  • Secondary bacterial infections – treat with ABX
  • Complications – otitis media, pneumonia, convulsions, encephalitis, haerring loss
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17
Q

Describe the rubella virus?

A

RUBELLA
• German measles, Togavirus, spread through sneezing/coughing
• Infective period 1 week before symptoms to 4 days after

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18
Q

How does rubella present? Mx? Complications?

A
Clinically:
• Rose-pink skin rash;
  - maculopapular
  - confluent: come together to form big blotches 
  - craniocaudal spread

Enanthema: petechiae on palate

Symmetrical lymphadenopathy,
Fever, coryza, arthralgia, conjunctivitis, serious risks in pregnancy

Management:
• No specific treatment – pass within 7-10 days, supportive – fluids, antipyretics

• Complications – haemorrhagic complications due to thrombocytopenia

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19
Q

How do we ivx rubella?

A

Serology - igm / igg (Elisa)

FBC - thrombocytopaenia

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20
Q

What is the pathogenesis of parvovirus B19?

A

Fifth disease/slapped cheek syndrome

  • Transmission via respiratory secretions, vertical transmission, transfusions
  • Infects erythroblastoid RC precursors in BM
  • Infectious period – 7-10 days before rash, 1 day after it develops
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21
Q

How does parvovirus present?

A

Can be asymptomatic

1 • Erythema infectiousum (most common) –
viraemic phase of fever, malaise, headache, myalgia with red rash on face (slapped cheek), progresses to maculopapular (lace) like rash in trunk and limbs;

reticular spread on extensor surfaces

Arthritis -
Typically involves the small joints of the hands, wrists, knees or ankles and is self-limited.

2 • Aplastic crisis – most serious consequence, occurs in children with chronic haemolytic anaemia (sickle cell, thalassaemia) or immunodeficient (infection persists 3wks+)

3 • Fetal disease – maternal transmission – leads to fetal hydrops, death due to severe anaemia

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22
Q

What is management for parvovirus?

A

usually self-limiting, supportive tx – fluids, analgesia

  1. Give paracetamol for the fever and arthralgia
  2. May need to add some NSAID; ibuprofen for the arthritis
  3. If Aplastic crisis : RBC transfusion
  4. If immunosuppressed - IVIG
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23
Q

How does ROSEOLA INFANTUM /Sixth disease present?

A

Very infectious

Caused by HHV6B sometimes HHV7

  • High fever (eg 40C) for 3-4 days
  • Rash AFTER fever stops

Discrete small rose-pink maculopapular rash/spots
starts on body/trunk and spread to arms, lasting 1-2 days

• Sore throat, lymphadenopathy

May have febrile seizures

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24
Q

Mx and complications of 6th disease?

A
  • Supportive, antipyretics, fluids

* Complications – high fever may cause febrile convulsions

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25
How does chicken pox present and what is the Mx? characterise rash
Pyrexia, headache, abdo pain, malaise • Crops of vesicles appear over 3-5 days – head, neck, trunk (sparse on limbs) – itchy • Macular -> Papular → vesicle → crust – several stages at once Management: • Supportive, fluids, minimise scratching, analgesia • Antihistamines, emollients for pruritis • Acyclovir if immunocompromised or severe Isolate/no school from 5 days from onset of rash OR till lesions gone/crrusted over
26
Aetiology, presentation and Mx of HAND, FOOT, MOUTH DISEASE?
* Most commonly due to Coxsackie A16 virus * Painful vesicular lesions on hands, foot, mouth, tongue, buttocks * Mild systemic features – fever, sore throat,spots in mouth → develop into ulcers * Disease subsides in a few days with fluids and analgesia do not need time off school (or isolation) - unless unwell
27
What are the causes of meningitis?
Viral are most common – most are self resolving • Bacterial may have severe consequences • Non-infectious causes: malignancy, AI disease
28
How does meningitis present?
• Lethargy, loss of interest, drowsiness, coma, seizures, muscle pains, resp symptoms, * Headache, photophobia, neck stiffness, +ve Kernig sign * Non-blanching purpuric rash - meningococcal rash (doesnt necessarily mean meningitis is yet present) ↑cap refill (>2) * ↑ICP: ↓consciousness, abnormal pupillary response, abnormal posturing * Late signs – papilloedema, bulging fontanelle in infants, opisthotonus (hyperextension of head and back)
29
How do we ivx meningitis?
Basic obs - HR, RR, O2 sats, BP, temp, AVPU Bacterial meningitis workup a lot more * FBC (often ↑WCC) * Blood glucose and blood gas (acidosis) * Coagulation, CRP, U&Es, LFTs (check for CMV, EBV, Coxiella) * Blood culture, throat swab, urine, stool culture, nasopharyngeal aspirate if resp symptoms * Rapid antigen test for organisms (on blood, CSF or urine) * LP for CSF (full workup; glucose, protein, cultre, gram stain) - if viral, most important test * PCR of blood and CSF (for N meningitides) * CXR as part of febrile work up * Consider CT (if focal neuro signs or ↓ consciousness) Treat as bacterial if → ↑CRP, ↑WCC, abnormal CSF
30
What are the contraindications of LP?
Contraindications for LP: signs of ↑ICP (fluctuating consciousness, bradycardia, focal neuro signs, abnormal posturing, unequal pupils, papilloedema
31
How do we manage meningitis?
Admit Bloods for culture then Empirical abx - based on age: • Immediate IV Ceftriaxone if > 3 months old • Use cefotaxime + amoxicillin/ampicillin if < 3 months old (abx is given to cover listeria) • Add vancomycin if recent travel outside the UK If signs of shock: Immediate fluid bolus of 20ml/kg saline over 5-10 mins If signs of shock persist, give a second bolus If signs persist after second bolus: • Give a first fluid bolus of 20ml/kg saline • Call for anaesthetic assistance for intubation and mechanical ventilation • Give vasoactive drugs: IV adrenaline or NA • Discuss with paediatric intensivist, consider more boluses Add 15 litre face mask O2 via reservoir breathing mask If > 3 months: give dexamethasone if suspected bacteria
32
How do we treat meningitis upon return of blood cultures?
Once cultures return: • Treat Hib with IV ceftriaxone for 10 days • Treat S pneumoniae with IV ceftriaxone for 14 days * Group B Strep with IV cefotaxime for > 14 days * L monocytogenes with IV amoxicillin/ampicillin (21), + gent (7) * G-ve bacilli with IV cefotaxime for 21 days
33
What further ivx should be done in meningitis? Why?
Offer a formal audiological assessment asap -Inflamm damage to cochlear Test for complement deficiency if: >1 episode of meningococcal disease, or meningococcal disease caused by serogroups other than B, or any serogroup plus a history of other recurrent or serious bacterial infections
34
Aseptic meningitis is caused by?
Viral meningitis most commonly
35
List some signs elicited in meningitis?
Kernig's sign - ___? | Brudzinski's sign -?
36
What is csf result in viral meningitis?
Protein - normal - high Glucose - normal - low WCC - high
37
What should we inform parents of when treating kid with IVIG for Kawasaki
IVIG affects the efficacy of live virus vaccines, i.e. MMR (measles, mumps, rubella) and varicella (chickenpox) vaccines, and these should be delayed for 11 months after the last dose of IVIG is given or repeated after 11 months if given earlier.
38
ENCEPHALITIS
ENCEPHALITIS
39
What is encephalitis? Aetiology?
Encephalitis is defined as inflammation of the brain parenchyma associated with neurological dysfunction Medical emergency It is the result of direct inflammation of the brain tissue, as opposed to the inflammation of the meninges (meningitis), and can be the result of infectious or non-infectious causes. An aetiological agent is only identified in around 50% of cases. Most common causes; Herpes viruses: herpes simplex virus (HSV)-1, HSV-2, varicella zoster virus VZV, cytomegalovirus CMV, Epstein-Barr virus EBV, human herpes virus-6 HHV6, herpes B virus HSV is a rare cause of childhood encephalitis but can be devastating long term consequences Others include: bacterial, fungi, parasites, paraneoplastic, prion
40
How does encephalitis present?
acute onset of a febrile illness and altered mental status; altered state of consciousness, seizures, personality changes, cranial nerve palsies, speech problems, and motor and sensory deficits. meningismus - sx ; neck stiffness, photophobia, headache without meningeal inflammation parotitis lymphadenopathy optic neuritis - inflamed optic nerve -> pain with eye movement n temporary vision loss. acute flaccid paralysis
41
How do we ivx encephalitis?
FBC Blood culture - reveals underline bacterial infection Ct head - hypodense lesions. Rest depends on aetiology MRI head - often hyperintense lesions. May see haemorrhage/ signs of oedema CSF culture n serology
42
How do we treat encephalitis?
Initially Viral - IV Aciclovir should be administered as soon as possible in all cases of suspected viral encephalitis. + supportive care ``` Once diagnosis confirmed: • If CMV suspected – add in ganciclovir and Foscarnet • If VZV – acyclovir/ganciclovir • EBV – acyclovir • Consider corticosteroids • Plus supportive care ``` Ongoing: Rehab
43
Complications of encephalitis?
Complications include seizures, hydrocephalus, and neurological sequelae (e.g., behavioural disturbances, motor problems).
44
How does toxoplasmosis present and rx?
Spiramycin started ASAP once maternal infection is diagnosed * 10% have clinical manifestations → retinopathy, cerebral calcification, hydrocephalus, long term neuro disabilities, convulsions, spasticity * Infected newborns can be treated with pyrimethamine and sulfadiazine for 1 year
45
Part 1 A mother presents to GP with maculopapular rash, fever, arthralgias, and lymphadenopathy. With more probing, she remembers she may have not completed her immunisations due to anti vax mother. When you ask her some gynae hx, her last period was 7 weeks ago. She does not use contraception. What tests would you do next?
UPT | Serology for rubella - IgM - ELISA
46
Part 2 UPT is +ve Serology confirms IgM rubella. What are next steps and
Notify the local Health Protection Unit Refer urgently to obstetrician for risk assessment and counselling Rubella Infection <8-10 week GA → 90% of congenital rubella syndrome → deafness, congenital HD, cataracts, mental retardation risk of spontaneous abortion No effective tx for rubella in pregnancy. Discuss risk to fetus and let her know recommendation will be for a termination (at gynae)
47
Case 2 p1 A 16 wk pregnant woman presents to triage with fever, malaise, myalgia, fatigue, arthralgia. She is a sex worker. O/E - generalised symmetrical macular, papular, or maculopapular diffuse rash, typically affecting the palms of the hands and plantar aspects of the feet. Also had generalised lymphadenopathy. She had all her vaccines in youth. What are your Dfx? What tests do you want?
Syphilis , as sex worker Serum Serology - syphilis Light field microscopy of swab of lesion
48
Case 2 p2 Results confirm syphillis. Sx suggest secondary phase Next steps?
Treat with IM benzylpenicillin (IV only in neurosyphillis) + prednisolone More than 1 month away from birth so kid should be okay If congenital syphilis suspected eg mum still positive around birth or reactive treponema test on infant serum - IV benzylpen for neonate for 10 days.
49
How does CMV present at birth? Complications?
Most common congenital infection → childhood disability and deafness neonate born with microcephaly, hearing loss, poor motor function Complications • When infant is treated; o 90% are normal with normal development o 5% have clinical features at birth – hepatosplenomegaly and petechiae with neurodevelopmental issues – sensorineural hearing loss o 5% develop problems later in life – mainly sensorineural hearing loss
50
How do we ivx and manage pregnant mum with cmv?
• Infection in mother is usually asymptomatic or causes a mild non-specific illness • Amniocentesis 6w after maternal infection to confirm vertical transmission • Close surveillance for US abnormalities – intracranial and hepatic calcification Deranged LFTs, pp65 antiginaemia.
51
How does CMV present?
infects the majority of humans. Primary infection in individuals with normal immune function is usually asymptomatic. After primary infection, CMV establishes a state of lifelong latency in various host cells, with periodic sub-clinical re-activations Can get fever, malaise, diarrhoea
52
39 weeks pregnant Mother is suspected to have chicken pox Within 5 days delivery. What precautions must now be taken?
Infants born in high risk period should also receive VZIG and prophylactic acyclovir Mum - oral acyclovir
53
List some Causes of collapsed baby on post-natal ward?
Sepsis !!! Cardiac Metabolic
54
What are some complications of chickenpox in neonate?
H
55
What is early onset sepsis in neonate? Aetiology?
Within 72 hours Bacteria from birth canal have ascended to invade amniotic fluid leading to secondary infection of foetus Can be caused by virus
56
How does neonatal sepsis present?
Respiratory distress, apnoea, temperature instability, altered behaviour, altered tone (floppy baby), feeding difficulties, jaundice, seizures, signs of shock, oliguria, hypo/hyperglycaemia, metabolic acidosis
57
How do we ivx neonatal sepsis?
Septic screen → FBC, U&Es, LFT, blood cultures, CRP (note will take 12-24 hours to rise) o Sepsis 6: OUT → blood culture, lactate/FBC, urine o IN → O2, IV abx, fluids LP if stable CXR
58
How do we manage neonatal sepsis?
Immediate abx – IV benzylpenicillin with gentamycin • Give for min 7 days, continue if still not recovered or micro advice • If meningitis suspected – IV amoxicillin with cefotaxime • Stop amoxicillin if later shown to be a G-ve infection
59
How does listeria infection present in mum and neonate?
bacteraemia, with mild, influenza-like illness in mother characteristically meconium staining of the liquor, widespread rash, septicaemia, pneumonia, meningitis
60
How does have present in neonate?
Presentation up to 4 weeks of age with → localised herpetic lesions on eye/skin, encephalitis or disseminated disease Rare but high mortality acyclovir to neonate if exposed
61
How do we treat neonate with hep B risk?
Infants of mothers who are HBsAg positive should receive hep B vaccination (active Ig and passive vaccination) within 12 hours after birth to prevent vertical transmission
62
How to rx malaria in kid?
Treatment – notify public health England ``` - Severe/complicated ▪ Artesunate or Quinine - Uncomplicated ▪ ACT – artemisinin combo therapy - Consider chloroquinine if not falciparum ```
63
How to rx typhoid in kid?
* Abx depending on region of travel | * Ceftriaxone, azithromycin or ciprofloxacin
64
How does dengue haemorrhagic fever occur?
Previously infected child has a subsequent infection with a different strain → severe capillary leak, hypotension, haemorrhagic manifestations
65
How do we ivx dengue? Mx?
FBC (low wcc), LFTs (high), serum albumin (low) • Gold standard – viral antigen, nucleic acid detection and serology * Supportive – fluids, monitoring* * Intensive care if established warning signs and increased deterioration * may need blood transfusion
66
If there is thrombocytopenia + dengue, this means?
Dengue HF
67
How does Dengue shock syndrome (DSS) present?
Has all the criteria of DHF plus circulatory failure as evidenced by: Rapid and weak pulse and narrow pulse pressure, or Age-specific hypotension and cold, clammy skin and restlessness.
68
Broadly what are the antibiotics we use by age group?
Under 2s - ampicillin (due to listeria) Then cefoxatime Above - ceftriaxone
69
How do T cell deficiencies present in childhood? rx?
Severe viral and fungal infections in first months of life: Oral thrush, Pneumocystis jirovecii PCP, Failure to Thrive HIV, SCID, Di George Rx: For T-cell and neutrophil defects: § Co-trimoxazole to prevent PCP § Itraconazole or fluconazole to prevent other fungal infections
70
How do B cell deficiencies present in childhood? rx?
Severe bacterial infections inc pneumonia Brutons agammaglobulinaemia Selective IgA deficiency HyperIGM syndrome CVID Rx: § Antibiotic prophylaxis (e.g. azithromycin) to prevent recurrent bacterial infections
71
How do neutrohphil deficiencies present in childhood? rx?
recurrent bacterial infections invasive fungal infections eg aspergillosis Chronic granulomatous disease For T-cell and neutrophil defects: § Co-trimoxazole to prevent PCP § Itraconazole or fluconazole to prevent other fungal infections
72
How do other immune deficiencies present in childhood?
Lecuocyte function/adhesion deficiency: - Delayed umbilical cord separation - delayed wound healing - chronic ulcers Complement deficiency: - Reccurent menningococcal - recurrernt bacterial - SLE like sx
73
what are the ways a child can catch HIV?
Major route is mother to child transmission – during pregnancy, at delivery and breast feeding Plus transmission by infected blood products, contaminated needles or sexual abuse (more uncommon)
74
how do we test for HIV in children?
• Children > 18 months old o HIV infection is diagnosed by detecting antibodies to the virus • Children < 18 months of age who are born to infected mothers → do HIV RNA PCR (standard) - All infants born to HIV positive mothers will be anti-HIV antibody positive for up to 18 months due to placental transfer of IgG - confirms exposure but not HIV infection • Two negative HIV RNA PCRs within the first 3 months of life, at least 2 weeks after completion of postnatal antiretroviral therapy, indicate the infant is not infected* *although this is confirmed by the loss of transplacental maternal HIV antibodies from the infant’s circulation after 18 months of age
75
How may AIDs present in a child?
If severe AIDS → opportunistic infections, Failure To Thrive, encephalopathy, malignancy
76
How do we mx HIV +ve infant?
1. Breastfeeding - triangle: - Can, IF viral load is very LOW, otherwise no! - if breast healthy: no bleeding/mastitis - if baby does not have upset tummy/diarrhoea - if 1 of above is missing -> formula 2• Anti-retroviral therapy (ART) o Babies born to HIV-positive mothers receive zidovudine monotherapy within 4 hours of birth for 6 weeks - DO HIV RNA PCR test If high risk eg mum wasnt following precautions in pregnancy, give: • Combinations of 3 (or 4) drugs are used Tenofovir + Lamivudine + Nevirapine (2 NRTI with an INSTI) • Test infants HIV RNA PCR at day 1, week 6 and week 12 of age • Confirmatory test at 18 months - these are to screen to see if HIV has been developed --------------------------- • Immunisations v important • EXCEPT do not give live vaccines eg BCG • Consider additional vaccination -> influenza, hep A/B, VZV • Prophylaxis with co-trimazole for PCP (if +ve PCR/ high viral load mum) MDT: infectious disease team, St Mary's family planning clinic (advice on breastfeeding etc) FU: weight, growth, adherence, symptoms
77
What is some advice breastfeeding mums with HIV should know?
1. Short&Sweet: Even if you are taking your HIV medication, your baby has double the chance of becoming infected with HIV if you breastfeed for 12 months rather than stopping before he or she is 6 months old. switch to bottlee/formula by 6 weeks. 2. Breast milk only If you choose to breastfeed, while your baby is under 6 months old, you should give breast milk only and no other food or drinks. This is known as exclusive breastfeeding. Giving breast milk and other foods may irritate the young (younger than 6 month old) baby’s tummy and increase the risk of HIV infection. http: //www.lhp.leedsth.nhs.uk/detail.aspx?id=177
78
when can HIV +ve mums have vaginal delivery?
Planned vaginal delivery can be offered ONLY if on HAART (so not just zidovudine monotherapy) and LOW viral load
79
what are some causes of secondary immune deficiencies?
Lymphoreticular malignancy. Drugs - particularly cytotoxic drugs and immunosuppressants. Viruses - eg, HIV. Malnutrition - the most common cause worldwide. Metabolic disorders - eg, renal disease requiring peritoneal dialysis. Trauma or major surgery. Protein loss - for example, due to nephrotic syndrome.
80
which immunodeficiency is known as 'bubble boy disease'?
SCID
81
Which genetic condition do we see Thymic aplasia -> genetic defects of the thymus and often the parathyroid glands and heart, associated with T-cell dysfunction and significant immune deficiency?
Di George
82
CVID puts you at increased risk of?
auto immunity
83
diagnostic criteria for immune dificiencies?
``` General: 4+ episodes Otitis in 1 year 2+ episodes pneumonia in 1 year FH immune deficiency Recurrent abscesses Adverse effect to BCG vaccine or other live vaccines Chronic diarrhoea (remember coealiac is autoimmune) Opportunistic infections ``` Condition specific: https://esid.org/Working-Parties/Clinical-Working-Party/Resources/Diagnostic-criteria-for-PID2