CHILD'S HEALTH 1 - Resp, ENT, Cardio, Gastro, Renal and GU Flashcards

Question

What is asthma?

Answer 1

This is a disease which is due to reversible airway bronchoconstriction, most often due to allergic stimuli.

Answer 2

Chest tightness Episodic Dyspnoea/SOB Wheeze Dry Cough (typically but can be wet)

Answer 3

No benefit from regular inhaled steroids Use bronchodilators May use oral steroids No interval symptoms and no excess of atopy

Answer 4

History * Cough after exercise or sometimes in the early morning, disturbing sleep. * Shortness of breath Spirometry * Peak expiratory flow rate (PEFR) **<80% predicted for height.** - **Peak flow rate (PEFR)**: measured multiple times a day over 2-4 weeks. **Variability of >20% is diagnostic** * FEV1/FVC <80% predicted. An improvement in FEV1 of 12% or more in response to either β2 agonists or corticosteroid treatment trials, together with an increase in volume of 200 ml or more, is considered diagnostic for asthma.

Answer 5

Under 5 yrs old Short-acting beta agonist – salbutamol Add a low dose corticosteroid inhaler Add leukotriene receptor antagonist (LTRA) - oral montelukast

Answer 6

Start a SABA – salbutamol as required Add a regular low dose corticosteroid inhaler - beclomethasone Add a LABA inhaler - salmeterol. Titrate up the corticosteroid inhaler to a medium dose. Oral leukotriene receptor antagonist - montelukast Increase the dose of the inhaled corticosteroid to a high dose.

Answer 7

Adrenal suppression! - Growth suppression - **(in the short term, but they should catch up)** Osteoporosis? - no evidence for an increased risk of fractures - but maybe slightly less bone density *Main factor for BMD in adult life is weight bearing exercise - which may be imparied in uncontrolled asthma* So on balance, pros often outweigh the cons in terms of risk - just measure growth for kids on ICS

Answer 8

Mild: SaO2 >92% in air, vocalising without difficulty, mild chest wall recession and moderate tachypnoea. Moderate: SaO2 <92%, breathless, moderate chest wall recession. Severe: SaO2 <92%, PEFR 33-50% best or predicted, cannot complete sentences in one breath or too breathless to talk/feed, heart rate >125 (over 5 years old) or >140 (2-5 years old), respiratory rate >30 (over 5 years) or >40 (2-5 years). Life-threatening: SaO2 <92%, PEFR <33% predicted, silent chest, poor respiratory effort, cyanosis, hypotension, exhaustion, confusion, A normal pCO2 When the patient has a high respiratory rate, one would expect a low CO2. Therefore, a high CO2 indicates exhaustion as they can no longer blow it off effectively.

Answer 9

Oxygen: SaO2 <94% should receive high flow oxygen to maintain saturations between 94-98%. Bronchodilators: Inhaled SABA (salbutamol) – via nebuliser if severe. *Inhaler and spacer device is as effective as nebuliser in children with mild/moderate asthma attack.* **Ipatropium bromide** (anti-muscuranic) added in if no or poor response to inhaled SABA Corticosteroids: **A short course (3 days) or steroids should be commenced. Oral prednisolone is first-line** *Steroid therapy should be given to all children who have an asthma attack, so for moderate asthma attacks give oral pred and salbutamol*

Answer 10

Kids under the age of 5. Commonly RSV or rhinovirus.

Answer 11

cold like symptoms, fever, runny nose and an expiratory bilateral wheeze. Generally not asthma if: Presenting < 3 years of age No atopic history Only occurs during viral infections

Answer 12

Airway: Oxygen Breathing Salbutamol inhalers or nebulisers Dexamethasone if mod-severe Circulation If severe/life-threatening for: IV access IV hydrocortisone IV Magnesium +/- IV Aminophylline +/- IV salbutamol

Answer 13

Persistent infantile wheeze tends to affect the small airways. It is associated with parental smoking or post-viral infection. ages 3-6 but also cold air, dust, animal dander and exercise. This has been called multiple-trigger wheeze. In the preschool age group, where a formal diagnosis of asthma may be unjustified, this distinction is helpful as many children in this group benefit from asthma preventer therapy and a significant proportion go on to have asthma.

Answer 14

No. Inhalers are unlikely to help; symptoms will improve as the child gets older.

Answer 15

This is a lower respiratory tract infection which leads to the ***blockage of small airway in the lungs*** – It is most seen in children younger than 2 and cases spike in autumn and winter **Commonest respiratory infection in infants** **can make v young children v ill**

Answer 16

**Most common – Respiratory Syncytial Virus (RSV)** Can be caused by Influenza, ADV prematurity, Being under the age of 2 Exposure to second hand smoke Downs syndrome or cystic fibrosis, Congenital heart disease or immunodeficiency.

Answer 17

Its a clinical diagnosis , dont need investigations Can do viral swab and MPA - not to diagnose, but so we can isolate kids or put kids w the same viruses in together

Answer 18

you can count too many ribs anteriorly . 7 here ! the chest is hyperinflated, as the kids are maxing out their lung spaces to try and get enough air in

Answer 19

General –> coryza, fever, irritability, poor feeding – **Dry cough** – Coryzal symptoms precede – **Wheeze** and crackles on auscultation – Respiratory distress –> chest wall recession, nasal flaring – May experience pauses in breathing Typical pattern of **worsening symptoms on day 3-4 days** of illness, but will get better within 3 weeks of supportive management grunting is a severe symptom A low-grade fever is typical in bronchiolitis

Answer 20

Bronchiolitis. Bronchiolitis affects the respiratory portion of the airway, where gas exchange takes place therefore you may see hypoxia and tachypnoea. Bronchitis affects the conducting portion of the airway and so is unlikely to have these effects. Typical pattern of worsening symptoms on day 3-4 days of illness

Answer 21

decreased sats, or needing oxygen enviroment or needing respiratory support Grunting Decreased feeding, as are in such difficulty feeding - hence that lots of babies w severe bronchiolitis will need **NG tube, - and so smaller, more frequent feeds**

Answer 22

Oxygenation - look at blood O2 sats levels Ventilation - Look at work of breathing, resp rate, most importantly **BLOOD GAS - LOOK AT CO2** *if you have poor ventilation, CO2 will be high*

Answer 23

Management: – This is largely supportive and based on symptomatic management and most cases self-resolve – Nutritional support, fluids, and oxygen therapy if low oxygen saturations SpO2 <92% –> admit to hospital if high flow o2 - needs to be humidified CPAP - delivers high pressure to keep you airways open BIPAP - used in neonates as they wont fight against the machine - gives alternating pressures, Mechanical ventilation Prevention: – Palivizumab (monoclonal antibody) (as immunisations) , can be given to infants who are at high risk of severe infection. This includes children with underlying heart/lung disease and premature babies.

Answer 24

Cystic fibrosis (CF) is an inherited, autosomal recessive, multi-system disease affecting mucus glands. Respiratory problems most prominent, as well as pancreatic insufficiency.

Answer 25

cystic fibrosis transmembrane conductance regulatory gene on **chromosome 7** **Δ-F508** is the most common mutation, where the codon for **phenylalanine** (F) in the CFTR gene is deleted, resulting in proteolytic degradation.

Answer 26

Misfolded CFTR protein **can't migrate from the endoplasmic reticulum to the cell membrane**, meaning there’s a lack of CFTR protein on the epithelial surface This means that it can’t pump chloride ions out, which means water doesn’t get drawn in, and the secretions are left overly thick.

Answer 27

- Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections, especially with: S. Aureus – flucloxacillin H. influenzae – amoxicillin Pseudomonas aeruginosa - ciprofloxacin

Answer 28

Thicker biliary secretions leads to an increased risk of biliary obstruction. Could lead to liver cirrhosis CFTR mutation can cause atrophy of the vas deferens leading to infertility

Answer 29

**Chronic cough w/Thick sputum production** Recurrent respiratory tract infections Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes Abdominal pain and bloating Most common cause of Meconium Ileus in infants Parents may report the **child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat** Poor weight and height gain (failure to thrive)

Answer 30

- Low weight - Nasal polyps - Finger clubbing - Crackles and wheezes - Abdominal distension

Answer 31

Other than **heel-prick/Guthrie test** *(looks for serum Serum Immunoreactive Trypsinogen.)* **Sweat test:** gold standard test; induce sweating *(by placing electrodes on skin)* followed by analysis of sweat to check Cl- concentration A result of **> 60 mmol/L (sweat chloride) is positive** and requires referral to a cystic fibrosis specialist (normal value < 40 mmol/Ll) Genetic testing: Genetic testing for CFTR gene mutation can be performed during pregnancy, via amniocentesis - **Lung function tests:** ***obstructive pattern seen***; and allows monitoring of treatment - **Sputum sample:** microbiological investigation during exacerbations Faecal elastase: test for pancreatic insufficiency

Answer 32

Exercise, no smoking! - **Airway clearance techniques:** minimum 2 times per day. Chest physiotherapy and postural drainage. - **Bronchodilator:** inhaled salbutamol for exacerbations rhDNase e.g. dornase alfa - an enzyme that breaks down material in secretions, making them less viscous - Nebulised hypertonic saline

Answer 33

personalised genetic treatments - targets specific mutation - **F508 del,** Lumacaftor is a CFTR corrector, acts as a **Pharmalogical chaperone** to bring the F508 del CFTR protein to the cell membrane Ivacaftor, a CFTR potentiator binds to channel directly, increases the chances of it opening. primarily the targets G551D mutation. Can be used in combination with one another

Answer 34

The bacteria enter from the back of the throat through the eustachian tube. Bacterial infection of the inner ear is often preceded by a viral upper respiratory tract infection. The anatomy of the eustachian tube in younger children is immature, typically being short, straight, wide (only becoming more oblique as the child grows), meaning infection is more likely.

Answer 35

Bacteria- Streptococcus pneumoniae (most common), haemophilus influenzae, and staphylococcus aureus Viral- Respiratory syncytial virus, rhinovirus, adenovirus

Answer 36

Otoscopy examination will reveal a **bulging**, red/cloudy tympanic membrane - **Ear pain** often associated with holding, tugging or rubbing of the ear in children - **Reduced hearing** - **Otorrhea** - discharge from ear - **Recent upper respiratory tract infection** - **Balance issues and vertigo:** if infection affects the vestibular system - **Non-specific symptoms** - Fever - Irritability and poor feeding - Vomiting - Sore throat

Answer 37

Will usually resolve within 3-7 days Can give amoxicillin for 5 days. 2nd line would be Co-amoxiclav Criteria for antibiotics - symptoms for 4 or more days, - systemically unwell, - immunocompromised, - < 2 years old with bilateral otitis media, - acute otitis media with perforation/discharge -**Emergency** symptoms of mastoiditis - ***tenderness behind the ear and the ear sticking out more prominently,**

Answer 38

Recurrent ear infections can lead to otitis media with effusion **(also called glue ear, Fig. 17.3c).** Children are usually asymptomatic apart from possible decreased hearing. The eardrum is seen to be dull and retracted, often with a fluid level visible.

Answer 39

A grommet keeps the middle ear aerated and prevents the accumulation of fluid in the middle ear.

Answer 40

1. Recurrent AOM. 2. Chronic otitis media + effusion. 3. ET dysfunction.

Answer 41

1. Glue ear. 2. Ear wax. 3. Otitis media. 4. Perforated ear drum.

Answer 42

Conductive hearing loss is usually ENT managed: - Wait and wait - most will resolve on their own. - Grommet insertion. - Temporary hearing aid.

Answer 43

Congenital infection, eg rubella, CMV Meningitis Genetics Consanguinity. Usher Syndrome hypoxic-ischemic injury to the brainstem, hemorrhage into the inner ear, toxic effects of bilirubin or aminoglycoside,

Answer 44

Sensori-neural hearing loss is often managed by a paediatrician. Treatments involve hearing aids or cochlea implants.

Answer 45

You would address the conductive problem first and then offer a hearing aid.

Answer 46

1. New-born hearing screen. 2. School entry hearing test. 3. Long term monitoring is done in high risk groups.

Answer 47

In periorbital cellulitis there is fever with erythema, tenderness, and oedema of the eyelid or other skin adjacent to the eye

Answer 48

Periorbital cellulitis should be treated promptly with intravenous antibiotics such as high-dose **ceftriaxone to prevent posterior spread** of the infection causing orbital cellulitis.

Answer 49

Squint - **a misalingment of the eyes, strabismus** Eyes not aligned - images in retina don’t match ---> get double vision. In kids, **as eye-brain connection isnt fully developed,** brain copes with this by reducing the signal from the less dominant eye --> As this eye becomes ignored, this becomes a lazy eye --> If untreated, becomes more and more disconnected from the brain == Called amblyopia

Answer 50

Concomitant (non paralytic) squints are due to **differences in the control of the extra ocular muscles.** The severity of the squint can vary. Paralytic squints are rare. They are due to **paralysis** in one or more of the extra ocular muscles.

Answer 51

Corneal light reflex test A Light in shone from a distance at both corneas simultaneously. If the light reflection does not appear in the same position in the two pupils, a squint is present Cover test The child is encouraged to look at a toy/light. If the fixing **(dominant)** eye is covered, the *squinting eye will move to take up fixation.** (Fig. 4.9). Cover test should be performed at different distances

Answer 52

Causes of paralytic squints - Infections like meningitis or encephalitis can damage the nerves - Birth trauma to the nerve - Nero disorders like cerebral palsy - hinder muscle control Tumours or growth

Answer 53

Atrail septal defect Ventricular septal defect Patent Ductus arteriosus

Answer 54

DA allows for blood to flow from pulmonary artery to aorata, bypassing lungs – In term infants, The Ductus arteriosis normally closes shortly after birth due to increased pulmonary flow (this enhances clearance of maternal prostaglandins which maintain duct patency) – But a failure of the ductus arteriosus to close results in left-to-right shunt between the aorta and the pulmonary artery, as the pressure is higher in the aorta than pulmonary artery – It is associated with prematurity and maternal rubella infection, downs syndrome

Answer 55

Continuous machinery like murmur, on left sternal edge large volume, bounding, collapsing pulse by a forceful upstroke followed by a rapid drop in systolic pressure, giving it a ‘water-hammer’ feel.

Answer 56

Symptoms: – Asymptomatic at birth with no cyanosis – However, if left untreated can lead to Eisenmenger syndrome and heart failure later in life – Continuous **‘machine-like’ murmur described as “rolling-thunder” with a left sub-clavicular thrill** – Wide pulse pressure – Heaving apex beat – Poor growth large volume, bounding, collapsing pulse by a forceful upstroke followed by a rapid drop in systolic pressure, giving it a 'water-hammer' feel.

Answer 57

– Indomethacin –> inhibits prostaglandin E synthesis causing closure of the ductus arteriosus – If very large or persistent, requires surgical repair indomethacin is given to the neonate in the postnatal period, not to the mother in the antenatal period, around 1 week after delivery

Answer 58

During development, a tissue called the septum primum between the left and right atria grows downward, slowly creating two separate chambers by closing the gap (ostium primum) *(know as the first opening)* The septum primum then fuses with the endocardial cushion and closes the ostium primum completely. A hole appears in the upper area of the septum primum, called the ostium secundum. *(known as the second opening)*

Answer 59

There is also the **septum secundum** *(an adjacent part of the septum)* which grows downward, just to the right of the septum primum. It covers the ostium secundum and leaves a small gap called the foramen ovale, thus creating a makeshift valve that allows blood to go from right atrium to left atrium, but not the other way

Answer 60

The types of atrial septal defect from most to least common are: - Ostium secondum defect. (most common) - Patent foramen ovale, where the foramen ovale fails to close (although this not strictly classified as an ASD). - Ostium primum,

Answer 61

Ostium secondum, where the septum secondum fails to fully close, leaving a hole in the wall. most common congenital heart condition ,in adults, (15% of all congenital heart defects) and 90% of ASDs

Answer 62

- Ostium primum, where the septum primum (first, left sided septum) fails to fully close, leaving a hole in the wall. Ostium primum = hole in septum primum, (ON THE LEFT - SO AFFECTS LEFT VENTRICLE ) This tends to lead to **atrioventricular valve defects making it an atrioventricular** septal defect. Found in 25% of down syndrome cases. (Less common then ostium secundum defect)

Answer 63

**Can often be asymptomatic in childhood, and first presentation in adulthood/late childhood can sometimes be stroke or heart failure or eisenmenger syndrome** - Signs - **Mid-systolic, crescendo-decrescendo murmur** loudest at the upper left sternal border - **Splitting S2 sound:** due to pulmonary valve closing after the aortic valve, as extra blood is flowing through it - **Cyanosis:** if reversal of shunt - Symptoms - **Dyspnoea** - **Difficulty feeding** - **Poor weight gain** - **Lower respiratory tract infections**

Answer 64

Ejection systolic, with **Splitting of the S2 sound**

Answer 65

- **Watch and wait:** for small ASD as it may spontaneously resolve - **Surgical closure of ASD** - Trans-catheter closure - Open heart surgery - **Anticoagulants:** used to reduce the risk of clots and stroke in adults

Answer 66

Ventricular Septal Defect When the ventricular septum has a gap in it. This can vary in size from tiny to the entire septum, forming one large ventricle.

Answer 67

Oxygenated blood then enters the left atrium and then the left ventricle. Here, blood travels from the high pressure LV to the low pressure RV (left to right shunt). The blood flowing through the VSD causes a holosystolic murmur At this point, patient's are acyanotic as there is no deoxygenated blood entering the systemic circulation.

Answer 68

Overtime, this causes increase of blood in Right sided circulation = **causing an increase in pressure.** Now **shunt is reversed** = **deoxygenated blood enters the systemic circulation** via the aorta and cause cyanosis - this is known as **Eisenmenger syndrome**. Causes Cyanosis at this point

Answer 69

- Signs - **Pansystolic murmur** at the lower left sternal border - May be a **systolic thrill on palpation** - **Cyanosis:** due to deoxygenated blood in systemic circulation - **Tachypnoea** - Symptoms - **Dyspnoea** - **Poor feeding** - **Failure to thrive** **. There are fine crepitations audible in both lung fields** - as VSD can cause pulmonary hypertension

Answer 70

A pansystolic murmur, on the lower left sternal boarder, louder in smaller defects A pansystolic murmur is one which last from the beginning S1 to the end of S2, and therefore obscures these heart sounds

Answer 71

Duiretics, like spironlactone Cardiac catherisation

Answer 72

- Hypoplastic left heart syndrome -*left side of the heart severely underdeveloped* * Critical aortic valve stenosis * Severe coarctation of the aorta * Interruption of the aortic arch - transposition of the great arteries - **Think - all lead to obstructed systemic circulation, so heart is fucked from the off - causes heart failure straight away**

Answer 73

Characteristics of an innocent ejection murmur include: soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area may vary with posture localised with no radiation no diastolic component no thrill no added sounds (e.g. clicks) asymptomatic child no other abnormality

Answer 74

- Large PDA, VSD or ASD - Anything that causes mixes of circulation

Answer 75

More due to acquired conditions: Rheumatic fever, cardiomyopathies, Eisenmenger syndrome

Answer 76

Shortness of breath – Inability to gain weight/poor growth – Predisposition to recurrent chest infections – **Hepatomegaly** – Signs of specific cause –> e.g., murmur, cardiomegaly, cyanosis

Answer 77

The underlying cause of heart failure must be treated. * Bed rest and nurse in semi-upright position: infants in chair/seat. * Supplemental oxygen **(not in left to right shunt).** * Diet: sufficient calorie intake. * Diuretics. * Angiotensin converting enzyme inhibitors

Answer 78

Tetralogy of Fallot transposition of the great arteries Eisenmenger Syndrome

Answer 79

Pulmonary stenosis **Right Ventricular hypertrophy** Ventricular septal defect Overriding aorta *It is the most common form of congenital cyanotic heart disease* *the severity of the right ventricular outflow tract obstruction (pulmonary stenosis) determines the degree of cyanosis and clinical severity*# PROVE Pulmonary stenosis Right Ventricular hypertrophy Overriding aorta Ventricular septal defect E = Ejection systolic murmur

Answer 80

TETROLOGY OF FALLOT - Pulmonary stenosis - **leads to RV hypertrophy** as it has to push harder to get blood through VSD - blood shunts from **right to left due to RV hypertrophy** - *Deoxygenated blood enters circulation* , ***get cyanosis*** Overriding Aorta - Entrance to aorta is further right then normal, above VSD - so more deoxygenated blood enters aorta when the RV contracts upwards Tetralogy of Fallot: the severity of the right ventricular outflow tract obstruction **(pulmonary stenosis)** determines the degree of cyanosis and clinical severity

Answer 81

YES! There is a greater pressure in the RV than the LV and so blood is shunted into the LV -> CYANOSIS! *the severity of the right ventricular outflow tract obstruction (pulmonary stenosis) determines the degree of cyanosis and clinical severity*

Answer 82

- **Neonates and babies**: typically manifests at around 1-2 months of life - **Family history of congenital heart disease** - **Rubella infection** - **Increased age of the mother** (over 40 years) - **Alcohol consumption in pregnancy** - **Diabetic mother** - **Down syndrome**: trisomy 21

Answer 83

**Ejection systolic murmur** in the pulmonary region (think due to pulmonary stenosis) Boot shaped heart, due to right ventricular hypertrophy

Answer 84

- Signs - **Ejection systolic murmur:** due to pulmonary stenosis - **Reduced SpO2,** particularly when distressed - **Respiratory distress** - **Cyanosis** - **Clubbing** - Symptoms - **Poor weight gain or ‘failure to thrive’** - **Difficulty feeding** - Hypercyanotic or ‘tet’ spells: **cyanosis, breathlessness and syncope**, particularly when crying or feeding - **Squatting posture**

Answer 85

Transposition of the great arteries is a condition where the attachments of the aorta and the pulmonary trunk to the heart are swapped (“transposed”). This means the right ventricle pumps blood into the aorta and the left ventricle pumps blood into the pulmonary vessels. In this scenario are two separate circulations that don’t mix: one travelling through the systemic system and right side of the heart and the other traveling through the pulmonary system and left side of the heart.

Answer 86

trick question - no murmur dickhead! – but**Gives a loud single S2**==> *pulmonary valve is flowing high pressure blood from the* ***left*** *ventricle* Loud S2! Egg on string appearance

Answer 87

Symptoms: – Cyanosis shortly after birth e.g., on day 2 when ductus arteriosus closes – The more mixing of blood there is, the less severe the presentation – **Gives a loud single S2** but **no murmur** ==> *pulmonary valve is flowing high pressure blood from the* ***left*** *ventricle* – If left untreated, leads to heart failure and mortality

Answer 88

Diagnosis: – Echocardiogram is the investigation of choice – Pulse oximetry –> shows reverse differential cyanosis (SpO2 in legs > SpO2 in arms), as the **legs can receive some oxygenated blood through the PDA.** In contrast, the **branches to the arms occur before the PDA** so they only get deoxygenated blood. – CXR –> shows **narrow upper mediastinum with an ‘egg on side’** appearance of the cardiac shadow

Answer 89

– Prostaglandin infusion (5mg/kg/min) and oxygen to manage acute cyanosis e administration of prostaglandins (such as alprostadil) to promote duct patency. – Surgical correction –> arterial switch procedure is the definitive treatment

Answer 90

Turners syndrome, bicuspid aortic valve, male sex

Answer 91

Radiofemoral delay Differential in blood pressure in upper and lower limbs (high in arms, low in Legs) Mid Ejection systolic murmur (similar to aortic stenosis) It's acyanotic Can often be seen with Turners syndrome

Answer 92

Low insertion of the tricuspid valve, meaning that the right atrium is too big and the right ventricle is too small - leads to poor flow to the pulmonary vessels Associated w Lithium use in pregnancy, ASD, PFO. WPW syndrome

Answer 93

very prominent murmur during systole and diastole, loudest over the left sternal edge. A chest X-Ray shows massive cardiomegaly, particularly prominent in the right atrium. Evidence of heart failure (e.g. oedema) **Gallop rhythm heard on auscultation** characterised by the addition of the third and fourth heart sounds: Severe tricuspid regurgitation and an enlarged right atrium raise right atrial pressure. When right atrial pressure exceeds left atrial pressure, deoxygenated blood flows from the right atrium to the left atrium through the ASD/PFO. **Cyanosis** Shortness of breath and tachypnoea Poor feeding Collapse or cardiac arrest

Answer 94

Rheumatic fever is caused by group A beta-haemolytic streptococcal, typically streptococcus pyogenes causing tonsillitis. The immune system creates antibodies to fight the infection. These antibodies also match antigens on the cells of the person’s body, eg in the heart. This results in a type 2 hypersensitivity reaction, where the immune system begins attacking cells throughout the body.

Answer 95

Improvements in sanitation, reduction in overcrowded living conditions, treatment of streptococcal pharyngitis with 10-day course of antibiotics and changes in streptococcal virulence have led to its virtual disappearance in developed countries.

Answer 96

The typical presentation of rheumatic fever occurs 2 – 4 weeks following a streptococcal infection, such as tonsillitis. Symptoms affect multiple systems, causing: Fever Joint pain Rash Shortness of breath Chorea Nodules Heart involvement, nervous system involvement

Answer 97

can lead to Carditis, or inflammation throughout the heart, with pericarditis, myocarditis and endocarditis, leads to: Tachycardia or bradycardia Murmurs from valvular heart disease, **typically mitral valve disease** Pericardial rub on auscultation Heart failure

Answer 98

Skin **Subcutaneous nodules** = Firm painless nodules occur over extensor surfaces of joints, such as the elbows. **Erythema marginatum** == **rash pink rings** of varying sizes affecting the torso and proximal limbs **Chorea** is the key nervous system symptom. This involves irregular, uncontrolled and rapid movements of the limbs. This is also known as Sydenham chorea and historically as St Vitus’ Dance.

Answer 99

Throat swab for bacterial culture **ASO antibody titres** Echocardiogram, ECG and chest xray can assess the heart involvement A diagnosis of rheumatic fever is made using the Jones criteria *Anti-streptococcal antibodies (ASO) are antibodies against streptococcus. They indicate a recent streptococcus infection and can be helpful in supporting a diagnosis of rheumatic fever.*

Answer 100

Two major, or one major and two minor, criteria plus supportive evidence of preceding group A streptococcal infection (markedly raised or rising ASO titre or positive rapid streptococcal antigen test or positive group A streptococcus on throat culture) Major Criteria: J – Joint arthritis O – Organ inflammation, such as carditis N – Nodules E – Erythema marginatum rash S – Sydenham chorea Minor Criteria: F Fever E ECG Changes (prolonged PR interval) without carditis A Arthralgia without arthritis R Raised inflammatory markers (CRP and ESR) JONES FEAR

Answer 101

Patients with clinical features of rheumatic fever should be referred immediately for specialist management. Management involves medications and follow up: NSAIDs (e.g. ibuprofen) are helpful for treating joint pain Aspirin (high dose) and steroids are used to treat carditis Prophylactic antibiotics (oral or intramuscular penicillin) are used to prevent further streptococcal infections and recurrence of the rheumatic fever. These are continued into adulthood. Monitoring and management of complications

Answer 102

Tonsillitis caused by streptococcus should be treated with **phenoxymethylpenicillin (penicillin V)** for 10 days.

Answer 103

VSD, coarctation of the aorta and PDA or if prosthetic material has been inserted at surgery.

Answer 104

The most common causative organism is α- haemolytic streptococcus (Streptococcus viridans). Bacterial endocarditis is usually treated with high-dose penicillin in combination with an aminoglycoside, giving 6 weeks of intravenous therapy and checking that the serum level of the antibiotic will kill the organism. **eg amoxicillin and gentamicin**

Answer 105

It is caused by inappropriate relaxation of the lower oesophageal sphincter as a result of functional immaturity. A predominantly fluid diet, a mainly horizontal posture and a short intraabdominal length of esophagus all contribute

Answer 106

- Children with cerebral palsy or other neurodevelopmental disorders - preterm infants, especially in those with bronchopulmonary dysplasia * following surgery for oesophageal atresia or diaphragmatic hernia.

Answer 107

Faltering growth from severe vomiting * Oesophagitis – haematemesis, discomfort on feeding or heartburn, iron-deficiency anemia * Recurrent pulmonary aspiration – recurrent pneumonia, cough or wheeze, apnoea in preterm infants * Dystonic neck posturing (Sandifer syndrome) * Apparent life-threatening events

Answer 108

Prescribe a 1-2 week trial of an alginate e.g. Gaviscon) is the first line management of GORD in breastfed infants, Prescribe a 1-2 week trial of feed thickener is the first line management of GORD in a bottle-fed baby. r. Prescribe a 4 week trial of omeprazole is the second-line management of GORD in a breastfed infant. In extreme cases, - surgical management - eg Nissen fundoplication, in which the fundus of the stomach is wrapped around the intra-abdominal oesophagus,

Answer 109

Hypertrophy (thickening) and therefore narrowing of the pylorus, leading to gastric outlet obstruction.

Answer 110

Vomiting - **projectile**, milk, straight after feeding. Weight loss. Visible gastric peristalsis and palpable mass on test feed. Will be non bilious vomit. Hirschsprung's disease gives bilious vomit Most commonly presents at 2-8 weeks of age

Answer 111

1. U+E. 2. Blood gas. 3. Ultrasound scan - hypertrophy of pyloric sphincter. - the key invesitgatiom

Answer 112

Metabolic Alkalosis - low K+ and Cl-. **High bicarbonate** H + HCO3 = CO2 + H20 Due to vomiting, H reduces, and so the equilibrium shifts to the left (above), to compensate for this change (Le Chatelier's principle i think). Therefore more bicarbonate is released into the blood as a biproduct of trying to get H+ ions up The baby has vomited up all the HCl and the kidneys go into overdrive - increased **K+ secretion.**

Answer 113

- IV fluids. - Repeat gases to monitor alkalosis. - Stop feeding to stop vomiting. - **Ramstedt pyloromyotomy** is used to manage pyloric stenosis. - *(This involves division of the hypertrophied muscle down to, but not including, the mucosa)*

Answer 114

Rotavirus Norovirus Campylobacter, E.coli, Salmonella and Shigella

Answer 115

Signs of dehydration: – Reduced urine output, lethargic, sunken eyes – However, they are haemodynamically stable (normal BP, normal capillary refill time, warm extremities) Signs of shock: – Reduced consciousness, pale skin, appears unwell – They will start to get haemodynamically compromised –> low BP, high HR, long CRT, cold extremities

Answer 116

Early shock - Normal BP - Tachycardia - Tachypnoea - Pale or mottled extremities - Reduced urine output Late Shock - Hypotensive - Bradycardia - Acidotic (Kussmaul breathing) - Blue extremities - Absent urine output

Answer 117

Management: The mainstay of management is to treat the dehydration according to severity of the child: – If no evidence of dehydration –> Continue breastfeeding/other milk feeds and encourage oral fluid intake – If clinical dehydration –> give oral rehydration salts and continue breastfeeding – If in shock –> Admit to hospital for IV fluid rehydration Start IV fluid resuscitation in children or young people with a **bolus of 10 ml/kg over less than 10 minutes**

Answer 118

Constipation is common issue in children, especially around the ages of 2-3 as children are being potty trained. – Common reasons include not eating high-fibre foods, drinking enough fluids or feeling anxious about something such as starting nursery or school

Answer 119

Less than 3 complete stools/week – Hard, stool like rabbit dropping (type 1 according to Bristol Stool chart) – Difficulty passing stool –> straining, abdominal pain that is relieved by defecation – Anal fissures – Soiling of underwear with diarrhoea –> this maybe a sign of overflow soiling

Answer 120

Constipation with overflow incontinence: The mass in the left iliac fossa likely represents fecal impaction. Explanation to Parents: The child's bowels are likely impacted with stool, leading to overflow diarrhoea. The sensation of needing to defecate is reduced due to chronic constipation.

Answer 121

Not passing meconium within 48 hours of birth (cystic fibrosis or Hirschsprung’s disease) Neurological signs or symptoms, particularly in the lower limbs (cerebral palsy or spinal cord lesion) Vomiting (intestinal obstruction or Hirschsprung’s disease) Abnormal anus (anal stenosis, inflammatory bowel disease or sexual abuse) Failure to thrive (coeliac disease, hypothyroidism or safeguarding) Acute severe abdominal pain and bloating (obstruction or intussusception)

Answer 122

Correct any reversible contributing factors, recommend a high fibre diet and good hydration Start laxatives (movicol is first line) Faecal impaction may require a disimpaction regimen with high doses of laxatives at first Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts.

Answer 123

Get kids to be active Increase fibre intake - fruits cereals Increase water intake encourage kids to be more active - sitting down can lead to constipation Just get kids to get sit on the toilet loads, throughtou the day

Answer 124

Acute appendicitis is an acute inflammation of the vermiform appendix, most likely due to obstruction of the lumen of the appendix.

Answer 125

Key indicators include a history of prolonged constipation, the inability to pass formed stool, and the presence of hard, compact stool in the rectum during a physical exam. Additionally, children may experience soiling, abdominal pain, and a loss of appetite.

Answer 126

Start laxatives (movicol is first line) Polyethylene glycol 3350 + electrolytes (movicol) Add a stimulant laxative if polyethylene glycol 3350 + electrolytes does not lead to disimpaction after 2 weeks. Faecal impaction may require a disimpaction regimen with high doses of laxatives at first Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts. Laxatives should be continued long term and slowly weaned off as the child develops a normal, regular bowel habit. Bulking laxatives lactulaose, movicol, Stimulant Senna, Sodium pico sulfate Ennemas Antegrade colonic enema creates a stoma to flush the colon with an enema NG tube

Answer 127

Normally occurs due to luminal obstruction As the appendix **continues to secrete mucus the fluid and mucus build up** = increasing pressure = makes appendix get bigger = and push on the visceral nerve fibres causing pain **Bacteria now trapped** and able to multiply resulting in **invasion of gut organisms into the appendix wall** This leads to oedema, ischaemia, necrosis, perforation and inflammation

Answer 128

- **Periumbilical pain** (referred pain) which migrates to the **right iliac fossa (McBurney’s point)** - **Low-grade fever:** > 38°C suggests alternative pathology e.g. mesenteric adenitis - **Reduced appetite and anorexia** - **Nausea and vomiting:** persistent vomiting is unusual - **Diarrhoea:** rare but may occur in pelvic appendicitis or due to a pelvic abscess

Answer 129

- **Right iliac fossa tenderness:** rebound tenderness (pain when pressure is taken off) or percussion tenderness (pain during percussion) suggests localised peritonism - **Guarding** - **Rovsing’s sign:** pain in the right iliac fossa is worsened by pressing on left iliac fossa - **Psoas sign:** pain is worsened by extending the hip - **Obturator sign:** pain is worsened by flexing and internally rotating the hip **Tachycardia, hypotension and generalised peritonism:** suggests perforation

Answer 130

Guarding - when abdominal muscles tense up Rebound tenderness - pain when releasing pressure on palpation

Answer 131

Fluids: - **Analgesia**: patients can be in considerable pain - Morphine, Paracetamol - **Antiemetics**: can be given for nausea and vomiting e.g. ondansetron - **Preoperative IV antibiotics** - Metronidazole 500mg/8h + cefuroxime 1.5g/8h

Answer 132

Prompt appendicectomy: laparoscopic Postoperative antibiotics

Answer 133

Mesenteric adenitis Mesenteric adenitis is inflamed lymph nodes within the mesentery. It can cause similar symptoms to appendicitis and can be difficult to distinguish between the two. It often follows a recent viral infection and needs no treatment

Answer 134

This is a herniation of the bowel content through the umbilicus. It is a common finding in neonates and usually asymptomatic

Answer 135

**Direct hernia:** hernia protrudes through the posterior wall of the inguinal canal. - Less common (20%) **Indirect hernia:** hernia protrudes through the deep inguinal ring and into the inguinal canal - More common (80%)

Answer 136

groin swelling and likely, the presence of risk factors. nausea, vomiting, constipation, abdominal pain/discomfort could indicate obstruction or strangulation an inguinal/inguino-scrotal mass that you cannot ‘get above’, is reducible when lying flat (goes back into abdomnal wall does not transilluminate, has a positive cough reflex.- *If the swelling expands upon coughing*

Answer 137

Most hernias can be successfully reduced by ‘taxis’ (gentle compression in the line of the inguinal canal) with good analgesia. Surgery can then be planned for a suitable time when any oedema has settled and the child is well. If reduction is impossible, emergency surgery is required because of the risk of compromise of the bowel or testis. In girls, sometimes the ovary can become incarcerated within a hernia.

Answer 138

A foreign pathogen triggers the immune system, **which are able to get through the gut wall due a defect in the epithelium** This triggers a large and uncontrolled immune response - **These immune cells invade deep into the mucosa and organise themselves into granulomas** This leads to ulcers forming throughout all layers, hence Crohn's being said to be **transmural**, and cobblestone in appearence **the ileum is the most commonly affected site of crohns**

Answer 139

Abdominal tenderness Fever Mouth ulcers Rectal examination will show blood, skin tags, fissures and fistulas eye problems - uveitis *Lethargy and general ill health without gastrointestinal symptoms can be the presenting features, particularly in older children. There may be considerable delay in diagnosis as it may be mistaken for psychological problems*

Answer 140

Weight loss Diarrhoea Abdominal pain (most common in RLQ where the ileum is as this is the site most affected by Crohns) *Lethargy and general ill health without gastrointestinal symptoms can be the presenting features, particularly in older children. There may be considerable delay in diagnosis as it may be mistaken for psychological problems*

Answer 141

**Faecal calprotectin** (released by intestines when inflamed) **C-reactive protein** is a good indication of current inflammation FBC: leukocytosis during a flare; anaemia due to vitamin B12, folate or iron deficiency **Stool sample** to rule out infectious diseases - **U&Es**: to assess for electrolyte disturbance and signs of dehydration - **Coeliac serology**: to exclude coeliac disease

Answer 142

Oral corticosteroids e.g. budesonide and prednisolone, as well as an **elemental diet** - pre-digested nutrients

Answer 143

**IV hydrocortisone** in severe flare ups Add **anti-TNF antibodies e.g. Infliximab** if no improvement Consider adding **other immunosuppressive drugs** Surgery – doesn’t cure disease

Answer 144

**azathioprine or methotrexate** to remain in remission if there are frequent exacerbations. *Thiopurine methyltransferase level should be measured before using*

Answer 145

Its a recurrent, inflammatory and ulcerating disease involving the mucosa of the colon In contrast to adults, in whom the colitis is usually confined to the distal colon, 90% of children have pancolitis. *(inflammation of the entire colon)*

Answer 146

Erythema Nodosum - inflamed subcutaneous fat (see picture) Pyoderma gangrenosum - rapidly enlarging, very painful ulcer. - Osteoporosis and arthritis - Clubbing - Uveitis - eye inflammation - Conjunctivitis *taken from netters illustrated human pathology*

Answer 147

- Diarrhoea - Blood and mucus in stool - Urgency and tenesmus (cramping rectal pain) - Abdominal pain: particularly in left lower quadrant - Weight loss and malnutrition - Fever and malaise during attacks

Answer 148

**Stool studies for infective pathogens** - rule out infection Faecal calprotectin: will be raised, helps differentiate between irritable bowel disease **pANCA antibodies** - often a feature of UC FBC - **may show leukocytosis, thrombocytosis, and anaemia** **LFTs** - check every 6 to 12 months for surveillance of primary sclerosing cholangitis. Lead pipe appearance on a barium enema - indicative of chronic UC

Answer 149

Colonoscopy - - Red and raw mucosa with widespread **shallow ulceration** - No inflammation beyond the submucosa, unless fulminant disease - **Pseudopolyps**: mucosa adjacent to ulcers is preserved, which has the appearance of polyps - *They are caused by mucosal repair after chronic inflammation.* - **Crypt abscesses** due to neutrophil migration through gland walls - **Goblet cell depletion**, with **infrequent** granulomas *picture taken from netters illustrated human pathology*

Answer 150

First line: Anti-inflammatories such **as Aminosalicylates (like mesalazine or sulfasalazine)** Second line corticosteroid (prednisolone) **(THINK SALAZINES)** Anti-TNF drugs - infliximab

Answer 151

Hospital admission + A **high-dose intravenous corticosteroid**, eg hydrocortisone severe UC can lead to toxic megacolon

Answer 152

PANPROCTOCOLECTOMY = Removal of the colon and rectum Left with ileostomy or or J-pouch this is where small intestine is used to make rectum severe UC can lead to toxic megacolon Surgery will be curative

Answer 153

Pseudopolyps **Crypt abscess** Goblet cell depletion, Infrequent granulomas Increased plasma cells in lamina propria

Answer 154

***Suspect in all with diarrhoea, weight loss & anaemia*** Steatorrhoea *(fat in stools)* & stinking stools - due to *Inability to properly absorb fat from diet* Anaemia - *Due to inability to absorb B12, folate, iron* Weight loss Fatigue & weakness Diarrhoea Bloating Nausea + vomiting Other, more subtle, modes of presentation – e.g. short stature, anaemia, abdominal pain and screening, e.g. children with diabetes mellitus, are now much more common.

Answer 155

Serum antibody testing IgA tissue transglutaminase (tTG), **Endomysial antibodies (EMAs),** **Deaminated gliadin peptides antibodies** (anti-DGPs), Very high sensitivity & specificity ***Need to be on a gluten diet for testing!!*** *However, in patients with IgA deficiency there is a risk of false negatives, as the total IgA count will remain low ---> So test total IgA count as well, and IgG version of anti-TTG or anti-EMA antibodies!!* T Cell mediated!

Answer 156

Endoscopically Required for definite diagnosis +ve Findings: Villous atrophy Crypt hyperplasia Increased epithelial WBCs Target cells and Howell Jolly Bodies

Answer 157

Lifelong gluten-free diet Avoid foods containing wheat, barley, rye, oats Gluten-free food are now more available Poor compliance is the main reason for recurrence Symptoms & serology used to monitor response & compliance Correct vitamin deficiencies Pneumococcal vaccine given Hyposplenism

Answer 158

The failure to gain adequate weight or achieve adequate growth during infancy and childhood. The modern British growth charts consist of nine centile lines (0.4th, 2nd, 9th, 25th, 50th, 75th, 91st, 98th and 99.6th). The distance between each centile line (2/3 of a standard deviation) is known as a centile space NICE defines faltering growth as weight that has fallen down 2 centile lines.

Answer 159

a fall across 1 or more weight centile spaces, if birth weight was below the 9th centile. a fall across 2 or more weight centile spaces, if birth weight was between the 9th and 91st centiles. a fall across 3 or more weight centile spaces, if birth weight was above the 91st centile. when current weight is below the 2nd centile for age, whatever the birth weight.

Answer 160

1. Inadequate calorie intake. 2. Malabsorption. 3. Inadequate retention. 4. Increased calorie requirements. 5. Inflammatory disease.

Answer 161

1. Impaired suck/swallow. - eg Cleft palate, cerebral palsy 2. Inadequate availability of food. 3. Psychosocial deprivation. 4. Exclusion diets e.g. veganism Infant difficult to feed – resists feeding or disinterested * Conflict over feeding, intolerance of normal feeding behaviour, e.g. messiness, throwing food around, leading to an early cessation of meals

Answer 162

1. Coeliac disease. 2. Pancreatic disease e.g. CF. 3. Liver disease. 4. Enteropathy e.g. infective causes - giardia. 5. Cows milk protein intolerance.

Answer 163

Vomiting e.g. severe GORD, pyloric stenosis.

Answer 164

1. Chronic illness e.g. CHD, CKD, CF. 2. Thyrotoxicosis. 3. Malignancy.

Answer 165

Marasmus is a deficiency of all macronutrients, and in it, the child has a wasted, wizened appearance (Fig. 13.15). Oedema is not present. Affected children are often withdrawn and apathetic

Answer 166

kwashiorkor is a type of malnutrition caused by protein deficiency. It mainly occurs in children who are **weaning off breast milk,** while marasmus can develop in infants.] If your **diet has a lot of carbohydrates and very little proteins**, you may develop kwashiorkor

Answer 167

Congential condition where there is an Absence of parasympathetic ganglion cells in the myenteric plexus It's complex web of neurones, ganglion cells, receptors, synapses and neurotransmitters. It is responsible for stimulating peristalsis of the large bowel.

Answer 168

Hirschsprung’s occurs when the parasympathetic ganglion cells do not travel all the way down the colon, and a section of colon at the end is **left without these parasympathetic ganglion cells.** The aganglionic section of **colon does not relax, causing it to becomes constricted.** This leads to loss of movement of faeces and obstruction in the bowel

Answer 169

Downs syndrome **Neurofibromatosis** Waardenburg syndrome (a genetic condition causing pale blue eyes, hearing loss and patches of white skin and hair) Multiple endocrine neoplasia type II

Answer 170

diagnosis varies significantly depending on the individual and the extent of the bowel that is affected. Delay in passing meconium (more than 24 hours) Chronic constipation since birth Abdominal pain and distention Vomiting (Billious) Poor weight gain and failure to thrive

Answer 171

Hirschsprung-associated enterocolitis (HAEC) it's inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease. It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. It is life threatening and can lead to toxic megacolon and perforation of the bowel. It requires urgent antibiotics, fluid resuscitation and decompression of the obstructed bowel.

Answer 172

Abdominal xray can be helpful in diagnosing intestinal obstruction and demonstrating features of HAEC. Rectal biopsy is used to confirm the diagnosis. The bowel histology will demonstrates an absence of ganglionic cells. Rectal examination may reveal a narrowed segment and withdrawal of the examining finger often releases a gush of liquid stool and flatus.

Answer 173

If Hirschsprung;s disease is suspected, a full thickness rectal biopsy should be performed as investigation. However, while this is being organised etc, bowel washouts should be performed to allow the baby to pass meconium, so this is the initial treatment Management is surgical and usually involves an initial colostomy of aganglionic section of the bowel, followed by anastomozing normally innervated bowel to the anus.

Answer 174

Intussusception describes the **invagination of proximal bowel into a distal segment.** It most commonly involves ileum passing into the caecum through the ileocaecal valve It's the most common cause of intestinal obstruction in infants after the neonatal period. Although it may occur at any age, the peak age of presentation is 3 months – 2 years of age.

Answer 175

Usually, no underlying intestinal cause for the intussusception is found, although there is some evidence that **viral infection leading to enlargement of Peyer’s patches** may form the lead point of the intussusception. Associated with HSP, and CF An identifiable lead point such as a **Meckel diverticulum or polyp** is more likely to be present in children over 2 years of age

Answer 176

– Abdominal pain during which infant characteristically will pull their legs up to the chest area, and abdo distension – Vomiting (which can be green in colour due to bile) – Blood-stained stool **(red-currant jelly stool)** – **Sausage-shaped mass in RUQ** – Can cause venous obstruction giving bowel necrosis and perforation

Answer 177

Investigation **ultrasound is now the investigation of choice and may show a target-like mass** An X-ray of the abdomen may show distended small bowel and absence of gas in the distal colon or rectum The abdominal film demonstrates a large soft tissue opacity ('sausage-shaped') in the left upper quadrant. .

Answer 178

Reduction with air insufflation is the treatment of choice for intussusception. It involves inflating air or Contrast or water to try and reduce the telescoping bowel and manual reduction of the bowel. Surgical reduction may be necessary if enemas do not work. Analgesia e.g. morphine. 3. IV fluids if shocked, as there is often pooling of fluid in the gut, which can cause hypovolaemic shock

Answer 179

Intestines don't rotate properly during fetal development, resulting in an abnormal arrangement of the bowel Feature in exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia May be complicated by the development of volvulus. Main symptom is billions vomiting

Answer 180

Diagnosis is made by upper GI contrast study and USS = shows a whirlpool sign. The correct management is Ladd's procedure, which involves the untwisting of the volvulus and the removal of Ladd's bands, resection of necrotic bowel if present and removal of the appendix, to prevent future operations.

Answer 181

Meckel's diverticulum is an outpouching or bulge in the lower part of the small intestine. Which contains ectopic gastric mucosa or pancreatic tissue

Answer 182

Tthe pouch releases acid like the stomach which over time can cause ulcers to develop in the small intestine. These can worsen and bleed which shows as blood in the poo. It can also leak the liquid inside the small intestine into the abdomen, leading to a serious infection called peritonitis. The pouch can also twist leading to a condition called volvulus. Twisting can cause the blood supply to the intestine to be cut off, which can be a life threatening condition. It can also fold inwards leading to a condition called intussusception.

Answer 183

Generally asymptomatic, but may present with bleeding (which may be life-threatening) or intussusception or volvulus: its most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years 1. Severe rectal bleeding, large amoutns, and painless 2. Intussusception. 3. Volvulus.

Answer 184

Surgical resection.

Answer 185

undiagnosed coeliac disease or excessive ingestion of fruit juice, especially apple juice. temporary cow’s milk allergy following gastroenteritis, when a trial of a cow’s milk protein free diet may be helpful. dysmotility of the gut (a form of irritable bowel syndrome) So rule out Coealiac and Cows milk allergy first

Answer 186

vomiting of bile, a greenish-yellow fluid made in the liver and stored in the gallbladder. Bile enters the intestines through the duodenum and is *typically absent from vomit* unless there is an **obstruction or abnormality beyond the stomach (in the small intestine).** See in - intestinal malrotation w Volvulus - Duodenal/jejunoileal atresia - hirschprungs disesase - meconium ileus - intussception - necrotising entercolitis

Answer 187

The term ‘colic’ is used to describe a common symptom complex that occurs during the first few months of life. Paroxysmal, inconsolable crying or screaming often accompanied by drawing up of the knees and passage of excessive flatus takes place several times a day.

Answer 188

It involves hypersensitivity to the protein in cow’s milk. This may be IgE mediated, in which case there is a rapid reaction to cow’s milk, occurring within 2 hours of ingestion. It can also be non-IgE medicated, with reactions occurring slowly over several days. **allergic (NOT INTOLERANCE) to the protein in cows milk, not the lactose!**

Answer 189

Abdominal pain, eczema, flatulence, bloody stools, diarrhoea or constipation General allergic symptoms in response to the cow’s milk protein: Urticarial rash (hives) Angio-oedema (facial swelling)

Answer 190

IgE-mediated CMPA has rapid reaction occurring within 2 hours of ingestion. (1 mark) Non-IgE-mediated CMPA has a delayed reaction, occurring >2 hours to days. (1 mark) IgE-mediated reactions can include anaphylaxis, while non-IgE reactions are more commonly gastrointestinal. (1 mark)

Answer 191

Skin prick or specific IgE antibody testing

Answer 192

Breast feeding mothers should avoid dairy products Replace formula with special hydrolysed formulas designed for cow’s milk allergy – If severe symptoms –> use amino-acid based formula (AAF) milk (elemental formulas) *Hydrolysed formulas contain cow’s milk, however the proteins have been broken down so that they no longer trigger an immune response*

Answer 193

When there is progressive fibrosis and obliteration of the extra-hepatic and intra-hepatic biliary tree. Chronic liver failure and death can occur within 2 years. - Down syndrome is a big risk factor OR CFC1 mutation

Answer 194

A **high levels of conjugated bilirubin** suggests the liver is processing the bilirubin for excretion (by conjugating it), but it is not able to excrete the conjugated bilirubin because it cannot flow through the biliary duct into the bowel. Biliary atresia presents shortly after birth with significant jaundice due to high conjugated bilirubin levels.

Answer 195

– Mild jaundice in the first few weeks of life – Cholestasis signs –> pale stools, dark urine – Normal birthweight which is then followed by poor levels of growth and appetite – Hepatomegaly with later splenomegaly (due to portal hypertension) – If left untreated signs of liver cirrhosis, as bile backs up in the liver and damages it

Answer 196

1. Measure transcutaneous bilirubin - conjugated bilirubin would be raised. 2. LFT's would be abnormal. Ultrasond - can see absent gall bladder 3. ERCP imaging would fail to outline a normal biliary tree.

Answer 197

Surgery to bypass fibrotic ducts. Nutrition and vitamin supplementation. 1st line is Kasai procedure - ( A loop of jejunum is brought up and directly attached to the liver, so bile drains directly from liver to intestines.) Ursodeoxycholic acid is routinely used in infants with biliary atresia after surgery. If unsuccessful or late presentation = liver transplant.

Answer 198

This is a rare disorder with increased risk in children with Down syndrome. The treatment is surgical, with a duodenoduodenostomy. An abdominal x-ray shows a “double bubble” sign

Answer 199

They are cystic dilatations of the extrahepatic biliary system They present with neonatal jaundice or, in older children, presentation is with abdominal pain, a palpable mass, jaundice, or cholangitis

Answer 200

ultrasound or magnetic resonance cholangiopancreatography. Treatment is by surgical excision of the cyst

Answer 201

Baby has intrauterine growth restriction, Jaundice, failure to thrive, itchy rash, dark urine and hepatomegaly.

Answer 202

Liver biopsy will show: Multinucleated giant cells & Rosette formation. This will both ↑unconjugated + ↑ conjugated bilrubin. With associated deranged LFT’s

Answer 203

Physiological Jaundice caused by two factors: Foetal haemoglobin has a shorter life span than adult haemoglobin and is constantly being broken down. The newborn liver (UDP enzymes) cannot cope with all of this bilirubin, and so it builds up in the blood. Breastfeeding Jaundice - breastfed babies are often more jaundiced, and for longer than bottle fed babies.

Answer 204

Infection (most common is herpes simplex) Metabolic disease Seronegative hepatitis Drug induced Neonatal haemochromatosis

Answer 205

Gastro-oesophageal reflux Feeding problems Infection: * Gastroenteritis * Whooping cough (pertussis) * Meningitis Infection – including pyelonephritis, septicaemia, meningitis Appendicitis

Answer 206

Intussusception * Malrotation * Volvulus * Adhesions * Foreign body – bezoar Plyoric stenosis * Atresia – duodenal, other sites **Hirschprungs disease**

Answer 207

Acute pyelonephritis is when the infection affects the tissue of the kidney. It can lead to scarring in the tissue and consequently a reduction in kidney function. Cystitis means inflammation of the bladder, and can be the result of a bladder infection.

Answer 208

**Fever** - *indeed, this may be the only symptom of a UTI so Always consider (and exclude) a urinary tract infection in a child with a temperature, unless there is a clear alternative source of infection* any child under 3 months with a fever - **admit to hospital!** Vomiting Lethargy Irritability Vomiting Poor feeding Urinary frequency

Answer 209

Fever Abdominal pain, particularly suprapubic pain Vomiting **Dysuria** (painful urination) Urinary **frequency** Incontinence

Answer 210

Any child under 3 months old with fever - admit to hospital!! Cant do urine dip as not reliable enough = , send a urine microscopy sample Threshold for doing a lumbar puncture is alot lower in young babies with fever, as their BBB is alot easier to penetrate Treat with broad spectrum antbx that can get through to blood brain barrier - eg 3rd gen ceph eg Ceftriaxone

Answer 211

<1 year, female sex, white children, uncircumcised boys <1 year, previous UTI, sexual abuse, bladder/bowel dysfunction, not breastfed, anatomical abnormalities (e.g. obstruction, posterior urethral valves, ureterocele, nephrolithiasis), immunosuppression, malnourishment, dysfunctional elimination syndrome (‘holding on’ to urine) , poor hygiene (e.g. frequent soiling, wiping back to front in girls)

Answer 212

MSU clean catch - recommended method urine collection pads catheter samples or suprapubic aspiration (SPA) Before SPA is attempted, ultrasound guidance should be used to demonstrate the presence of urine in the bladder. Do not delay treatment in seriously ill infants Can't do dipstick in under 3 months - not accurate enough - send straight to the labs

Answer 213

Nitrites - *Positive result useful as very likely to indicate a true urinary tract infection (UTI) But some children with a UTI are nitrite negative* Leucocyte esterase stick testing (for white blood cells) - May be present in children with UTI but may also be negative Present in children with febrile illness without UTIs Positive in balanitis and vulvovaginitis If both postive, regard as UTI if one postive and one negative, Start antibiotic treatment if clinical evidence of UTI Diagnosis depends on urine culture If both negative - UTI unlikely.

Answer 214

E. coli – most common bacteria Proteus – more common in boys Pseudomonas – may indicate structural abnormality

Answer 215

Atypical UTI includes: * seriously ill or septicaemia * poor urine flow * abdominal or bladder mass * raised creatinine * failure to respond to suitable antibiotics within 48 hours * infection with atypical (non-E. coli) organisms. * Boys (more likely to have posterior urethral valves)

Answer 216

developmental anomaly of the vesicoureteric junctions. The ureters are displaced laterally and enter directly into the bladder rather than at an angle, where urine has a tendency to flow from the bladder back into the ureters. Can lead to frequent UTIs and renal scarring - which in turn can lead to CKD and hypertension

Answer 217

DMSA (Dimercaptosuccinic Acid) Scan - Inject radioactive material (DMSA) and use a gamma camera to assess how well the material is taken up by the kidneys. looks for **renal scarring** in a child with vesicoureteric reflux Micturating Cystourethrogram (MCUG) - catheterising the child, injecting contrast into bladder and see whether the contrast is refluxing into the ureters. **This is necesscary to diagnose VER

Answer 218

Horseshoe kidney Duplex kidney (two ureters from one kidney) Solitary kidney

Answer 219

The diagnosis of acute pyelonephritis is made if either there is: A temperature greater than 38°C Loin pain or tenderness This is a very important point to note, as it affects the way you would investigate the child for recurrent infections.

Answer 220

Infants younger tahn 3 months - 2-4 IV cefuroxime (1st gen Ceph, cant get through BBB so need to rule out meningitis). followed by oral - full septic screen, bloods, cultures, lactate Systemically well children - Treat with 3 days oral antibiotics. Advised to return if no better at 24-48hrs for reassessment - **Trimethoprim** Systemically unwell - (Fever > 38 +/- loin pain / tenderness) Treat with 7-10 days oral antibiotics (Consider IV antibiotics according to clinical judgement. Use lower threshold for IV antibiotics in younger children, those with significant risk factors and severely ill.)

Answer 221

E coli Extended spectrum ß-lactamase producer (ESBL) Resistant to all penicillins and cephalosporins Change to **meropenem** for serious infections Fosfomycin For uncomplicated UTIs: Fosfomycin is often a good choice for oral therapy in cases of cystitis caused by ESBL-producing E. coli. It is typically given as a single dose. 3. Nitrofurantoin For uncomplicated cystitis only: Effective for lower urinary tract infections, but it should not be used for pyelonephritis or systemic infections.

Answer 222

Cystitis - oral antibiotic treatment should be started with trimethoprim (if there is low risk of resistance), or nitrofurantoin Acute Pyelonephritis - oral antibiotic treatment should be started, with either cefalexin, or co-amoxiclav.

Answer 223

Primary nocturnal enuresis is where the child has never managed to be consistently dry at night. It is normal There is a genetically determined delay in acquiring sphincter competence, with two-thirds of children with enuresis having an affected first-degree relative.

Answer 224

Reassure parents of children under 5 years that it is likely to resolve without any treatment Lifestyle changes: reduced fluid intake in the evenings, pass urine before bed and ensure easy access to a toilet Encouragement and positive reinforcement. Avoid blame or shame. Punishment should very much be avoided. Treat any underlying causes or exacerbating factors, such as constipation Enuresis alarms Pharmacological treatment - **Desmopressin** (vasopressin analogue)

Answer 225

a child wetting the bed when they have previously been dry for at least 6 months before Urinary tract infection Constipation Type 1 diabetes New psychosocial problems (e.g. stress in family or school life) Maltreatment

Answer 226

MOST COMMON IN CHILDREN *Reduced blood flow to kidney leading to a falling GFR* **Shock, + Hypovolaemia (fluid loss)** - this could be due to burns, sepsis, acute haemorrhage *trauma or bleeding*, anaphylaxis - Decompensated Liver disease- Hepatorenal syndrome GI losses – diarrhoea and vomiting, **Gastroenteritis**

Answer 227

Vascular: **– haemolytic uraemic syndrome** - most common type of renal – vasculitis – embolus * Tubular: – acute tubular necrosis – ischaemic – toxic - drugs * Glomerular: – glomerulonephritis * Interstitial: – pyelonephritis

Answer 228

NSAIDs – inhibits COX which causes excess vasoconstriction of afferent arteriole Aminoglycosides - eg gentamicin – 10-15% incidence of ATN ACE-I/ARBs – results in dilated efferent arterioles decreasing GFR Methotrexate Gentamicin ***DAMN = (diuretics, ACEi/ ARBs, metformin, NSAIDs)*** Loop diuretics are considered to be more harmful for kidneys than Thiazides and K+ sparing ones

Answer 229

Obstruction: – congenital, e.g. posterior urethral valves *in boys, urethra is blocked* – acquired, e.g. blocked urinary catheter

Answer 230

Liver cirrhosis leads to portal hypertension In response to this, **portal blood vessels dilate,** stretched by large amounts of blood pooling there. This leads to a loss of blood volume elsewhere, including kidneys. Leads to **hypotension in the kidney and activation of the RAAS.** This causes renal vasoconstriction, which combined with low circulation volume leads to starvation of blood to the kidney *Hepatorenal syndrome is fatal within a week or so unless liver transplant is performed*

Answer 231

circulation and fluid balance meticulously monitored urgently corrected with fluid replacement and circulatory support

Answer 232

3 THINGS - 1. **IV stat of Calcium Gluconate** - Stabilisation of the myocardium 2. **Variable rate insulin with dextrose infusion - to increase cellular uptake of potassium** = Reduction of serum potassium 3. **oral calcium resonium** - Reduction of total body potassium - treat underlying cause

Answer 233

hyperphosphataemia: Calcium carbonate Dietary restriction Metabolic acidosis Sodium bicarbonate

Answer 234

Treat underlying cause - **Regular monitoring:** fluids, urine output, daily weights, baseline creatinine, serial U&Es - **Cease nephrotoxic drugs** - Hypovolaemia: **IV fluids** - Hypervolaemic: **fluid restriction and diuretics e.g. furosemide** - Obstruction: **catheter insertion; surgery to relieve obstruction** ***TREAT COMPLICATIONS*** - Hyperkalaemia, pulmonary oedema, uraemia, acidaemia - **Bicarbonate**

Answer 235

Dialysis – removal of toxic products in body for patients with failed kidneys. refer to critical care, get specialist involved!! Use of a vasopressor, ie **Noradrenaline** Consider the need for an inotrope **(e.g., dobutamine)** to optimise cardiac output if kidney hypoperfusion is caused by impaired cardiac function due to poor left ventricular systolic function

Answer 236

1 >90 Normal or increased GFR with other evidence of renal damage* 2 60–89 Slight decrease GFR with other evidence of renal damage* 3 A - 45–59, 3 B - 30–44 = Moderate decreased GFR with or without evidence of other renal damage 4 15–29 Severe decreased GFR with or without evidence of renal damage 5 <15 Established renal failure

Answer 237

anorexia and lethargy * polydipsia and polyuria * faltering growth/growth failure * bony deformities from renal osteodystrophy (renal rickets) * hypertension * acute-on-chronic renal failure (precipitated by infection or dehydration) * incidental finding of proteinuria * unexplained normochromic, normocytic anaemia

Answer 238

Improve nutritional intake with calorie supplements Salt and bicarbonate supplements phosphate restriction and activated vitamin D to prevent renal osteodystrophy recombinant human EPO, and Growth hormone Diaylsis and transplantation

Answer 239

Nephrotic syndrome – protein leaks due to inflammation of podocytes Acute GN (nephritic syndrome) – blood vessels inflamed 🡪 blood leaks out Rapidly progressive GN – features of acute nephritis, focal necrosis and rapidly progressing renal failure

Answer 240

Defined as an increase in interstitial fluid Obstruction of lymphatic drainage - Congential Lymphoedma - Hyperplasia of lymph nodes leads to a block * Obstruction of venous drainage - DVT * Lowered oncotic pressure (low albumin) * Salt and water retention (heart failure and CKD )

Answer 241

Nephritic syndrome can be differentiated from nephrotic syndrome because the proteinuria is generally under 3.5 grams per day, or within the “sub-nephrotic range”.

Answer 242

Post-streptococcal glomerulonephritis (PSGN) is usually an immunologically-mediated **delayed consequence of pharyngitis or skin infections** caused by streptococcus pyogenes

Answer 243

- Most frequently seen in children - Usually develops 1-2 weeks after URTI (upper respiratory tract infection)

Answer 244

Haematuria Recent strep infection

Answer 245

- **Bloods:** low levels of C3 and CH50 as C3 has been deposited in kidney FBC - mild normchronmic and normocytic anaemia raised antiDNAse B Incresased Urea, hyperkalaemia - **Positive streptozyme test** confirms recent group A streptococcal infection Macroscopic haematuria

Answer 246

Supportive. - **Furosemide:** for initial treatment of hypertension - **Antibiotics**

Answer 247

It is a type III hypersensitivity reaction where immune complexes are deposited in various places including kidney Most common site of deposition is in the subendothelial space of the glomerular basement membrane Can also be in the Bowerman's space, Basement membrane, and in Mesangial cells.

Answer 248

Cells have faulty DNA, and when these faulty cells undergo apoptosis, these are released and become **nuclear antigens** immune cells recognise them as forgein and create **antinuclear antibodies that bind to them.** These can travel in the blood and get stuck in lungs, joint, skin, CNS, Heart Kidneys etc

Answer 249

Anti DNAase B

Answer 250

Anti dsDNA, antinuclear (ANA) and anti-smith

Answer 251

Similar to IgA Nephropathy. **IgA vasculitis, also known as Henoch-Schonlein purpura**: **the difference is IgA nephropathy only affects the kidneys**, while IgA vasculitis can cause nephritic or nephrotic syndrome, and also presents with colicky abdominal pain, bloody stool, arthritis, and palpable skin lesions, hyperteniosn and haematuria

Answer 252

**On electron microscopy**: immune complexes are seen in the mesangium. - **On immunofluorescence:** IgA immune complexes in the mesangium

Answer 253

characterized by glomerulonephritis, end-stage kidney disease, and hearing loss Blood in urine is universal. Proteinuria is a feature as kidney disease progresses Alport syndrome is caused by an inherited defect in type IV collagen—so this is why Alport syndrome affects different seemingly unrelated parts of the body (ears, eyes, kidneys, etc.).

Answer 254

Alport syndrome is caused by mutations in COL4A3, COL4A4, and COL4A5, three of six human genes involved in basement membrane (type IV) collagen biosynthesis. In most people with Alport syndrome (about 85%), the condition is inherited in an X-linked pattern,[19] due to mutations in the COL4A5 gene. Can also be inherited as autosomal recessive, on chromosome 2

Answer 255

presents in first 3 months of life, very rare Recessively inherited, more common in consanguineois families leads to severe hypoalbuminaemia unilateral nephrectomy may be necessary for its control, followed by dialysis for stage 5 (most severe) chronic kidney disease, which is continued until the child is no longer nephrotic and old enough for renal transplantation.

Answer 256

caused by T-cells in the blood, that **release cytokines-glomerular-permeability factor**, that specifically damages the foot processes of the podocytes, **making them flatten out - a process called effacement.** Damaged foot processes **lose their negatively charged coat, eventually allowing negatively charged molecules**, **MOST COMMONLY SEEN IN CHILDREN**

Answer 257

- allowing negatively charged molecules, like albumin, to slip into the nephron. - Even though albumin goes through, ***other larger proteins like immunoglobulins*** don't. - **So there's selective proteinuria;** in contrast to other causes of nephrotic syndrome which are characterised by **non-selective proteinuria.**

Answer 258

If you spot a 2 – 5 year old child with oedema, proteinuria and low albumin, you may be asked about the underling cause. The answer is likely to be nephrotic syndrome.

Answer 259

Management – corticosteroids ± cyclophosphamide or cyclosporine (for frequent relapsing cases) , or DMARDs 60mg/m^2 per day - work out child's body surface area Normally respond very well to steroids. 30% single relapse, 30% frequent relapse, 30% will become steroid resistant

Answer 260

Proteinuria: >3.5 g/24 hours or urine protein/creatinine ratio >2. Hypoalbuminemia: Serum albumin <2.5 g/dL. Edema. Hyperlipidemia.

Answer 261

Hypospadias is a condition affecting males, where the urethral meatus (the opening of the urethra) is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum. This might be further towards the bottom of the glans (in 90% of cases), halfway down the shaft or even at the base of the shaft. There can also be an associated condition called chordee, where the head of the penis bends downwards. Cryptorchidism (undescended testes) is present in around 10% of patients with hypospadias

Answer 262

Hypospadias requires referral to a paediatric specialist urologist for ongoing management. It is important to warn parents not to circumcise the infant until a urologist indicates this is ok. Mild cases may not require any treatment Surgery is usually performed after 3 – 4 months of age Surgery aims to correct the position of the meatus and straighten the penis

Answer 263

HUS is a triad of acute renal failure **(AKI)**, microangiopathic **haemolytic anaemia**, and **thrombocytopenia** cuased by the toxins released by bacteria following gastroenteritis It causes thrombosis in small blood vessels - *one of the thrombotic microangiopathies*

Answer 264

E. coli O157 or Shigella release Shiga toxins, that can pass out of the gut and enter the bloodstream. most common is E. coli O157

Answer 265

Microangiopathic haemolytic anaemia (MAHA) involves the destruction of red blood cells (haemolysis) due to pathology in the small vessels (microangiopathy). Tiny blood clots (thrombi) partially obstruct the small blood vessels and churn the red blood cells as they pass through, causing them to rupture. would see Schistocytes (1 mark) or helmet cells (1 mark)

Answer 266

E. coli O157 and Shigella cause gastroenteritis. Diarrhoea is the first symptom, which turns bloody within 3 days. Around a week after the onset of diarrhoea, the features of HUS develop: **Fever Abdominal pain** Lethargy Pallor Reduced urine output (oliguria) **Haematuria** Hypertension Bruising **Jaundice (due to haemolysis)** Confusion

Answer 267

Stool culture to establish the causative organism. HUS is a medical emergency and requires hospital admission and supportive management with treatment of: Hypovolaemia (e.g., IV fluids) Hypertension Severe anaemia (e.g., blood transfusions) Severe renal failure (e.g., haemodialysis) It is self-limiting, and most patients fully recover with good supportive care.

Answer 268

if picked up at 6-8 weeks - refer in 3 months Unilateral undescended testis referral should be considered from around 3 months of age, with the baby ideally seeing a urological surgeon before 6 months of age orchidopexy: Surgical practices vary although the majority of procedures are performed at around 1 year of age

Answer 269

Bilateral undescended testes Should be reviewed by a senior paediatrician within 24hours as the child may need urgent endocrine or genetic investigation

Answer 270

The major risk factors for SIDS are: prone sleeping parental smoking bed sharing hyperthermia and head covering prematurity

CHILD'S HEALTH 1 - Resp, ENT, Cardio, Gastro, Renal and GU Flashcards

(294 cards)