Paeds 1 🫁 🫀🚽 Flashcards

Question

what causes chronic kidney disease? (4)

Answer 1

``` hypertension diabetes glomerular sclerosis lupus RA HIV NSAIDS toxins - tobacco ```

Answer 2

``` nausea loss of appetite encephalopathy (tremor) pericarditis increase bleeding uremic frost - crystals in skin ```

Answer 3

less potassium is excreted which leads to build up in blood | hyperkalemia - can cause arrhythmias

Answer 4

low - hypocalcemia | kidneys activate vitamin D which aids the absorption of calcium

Answer 5

parathyroid hormone is released which causes the bones to lose calcium this leads to bone being brittle which is called renal osteodystrophy

Answer 6

changes in GFR less than 60ml/kg/1.73m2 Proteinuria (ACR >3mg/ml) kidney biopsy to look for changes such as glomerular sclerosis.

Answer 7

proteinuria (>3+ on dipstick) hypoalbuminaemia oedema

Answer 8

minimal change disease can also be caused by focal glomerulosclerosis and post stept nephritis

Answer 9

there is damage and inflammation to the podocytes which normally help prevent protein loss from the glomerulus. As a result of this, increased protein is lost through the nephron tubules.

Answer 10

90% of proteinuria resolves with corticosteorid therapy (prednisolone for 4 weeks then a smaller dose every other day for 4 weeks)

Answer 11

hypovolaemia thrombosis infection hypercholesteroleamia

Answer 12

nearly always presents as nephrotic syndrome

Answer 13

the majority are idiopathic drugs - NSAIDS, rifampicin Hodgkin's lypmhoma, thymoma infectious mononucleosis

Answer 14

Oedema Proteinuria Hypoalbuminaemia Hyperlipidemia

Answer 15

80% of cases are steroid responsive | cyclophoshphamide in the next step for steroid-resistant cases

Answer 16

when nephrotic syndrome presents within the first 3 months of life. it is rare commonest kind is recessively inherited associated with high mortality usually due to complications of hypoalbuminaemia

Answer 17

post infectious (including streptococcus) Vasculitis (HSP, SLE, wegener granulomatosis) IgA nephropathy and mesangiocapillary glomerulonephritis anti-glomerular basement membrane disease (good pasture syndrome)

Answer 18

decreased urine output and volume overload hypertension which may cause seizures oedema, characteristically around the eyes haematuria and proteinuria

Answer 19

water and electrolyte balance and the use of diuretics when necessary

Answer 20

``` characteristic palpable purpuric rash on buttocks and extensor surfaces of arms and legs arthralgia periarticular oedema abdominal pain glomerulonephritis ```

Answer 21

``` between 3 and 10 years more common in boys peaks during winter months often preceded by a response infection cause is unknown ```

Answer 22

it is a familial nephritis usually an X-linked recessive disorder that progresses to end-stage renal failure by early adult life in males and is associated with sensorineural deafness and ocular defects.

Answer 23

congenital abnormality of the penis which occurs in approx 3/1000 male infants an abnormal opening below the urethra can also happen in girls but way way more common in boys

Answer 24

a ventral urethral meatus a hooded prepuce chord (ventral curvature of the penis) in more severe form the urethral meatus may open proximally in the more severe variants

Answer 25

corrective surgery is performed before 2 years of age

Answer 26

Urine dipstick: proteinuria and check for microscopic haematuria MSU to exclude urinary tract infection. Quantify proteinuria using an early morning urinary protein:creatinine ratio or albumin:creatinine ratio. FBC and coagulation screen Urea and electrolytes CXR

Answer 27

bloods (raised IgA, raised ESR, ASO titre (check for PSG) | urine dip

Answer 28

Supportive care- rest, rehydration, analgesia for MSK pain | prednisolone if severe

Answer 29

URTI or pharyngitis then 1-3 weeks later haematuria oliguria proteinuria odema hypertension signs of CV overload

Answer 30

``` bloods - FBC - anaemia - U&Es - increased urea and creatinine, increased potassium, acidosis - antistreptolysin O increased anti DNAase B titre low C3 urinalysis - haematuria, proteinuria renal biopsy ```

Answer 31

penicillin - 10 days treat hypertension and fluid overload correct metabolic imbalances

Answer 32

there is abnormal back flow of urine from the bladder to the ureter and kidneys there is abnormal vesicoureteric junction where the ureters enter the bladder - the ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle therefore shortened ureter, and the junction cannot function adequately

Answer 33

I - Reflux into the ureter only, no dilatation II -Reflux into the renal pelvis on micturition, no dilatation III-Mild/moderate dilatation of the ureter, renal pelvis and calyces IV - Dilation of the renal pelvis and calyces with moderate ureteral tortuosity V - Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity

Answer 34

micturating cystourethrogram for diagnosis (MCUG) | DMSA (dimercapto succinic acid) scan should also be performed to check for scarring (4-6 months after illness) Management: Avoid constipation Avoid an excessively full bladder Prophylactic Abx Surgical input from paediatric urology

Answer 35

acute renal failure microangiopathic haemolytic syndrome thrombocytopenia

Answer 36

secondary HUS | classically caused by shiga toxin producing E.coli (STEC)

Answer 37

primary HUS due to complement dysregulation

Answer 38

FBC (anaemia, thrombocytopenia, fragmented blood film) U&E (acute kidney injury) stool culture (Shiga toxin-producing Escherichia coli) LDH (non specific sign for haemolytic anaemia)

Answer 39

supportive treatment - fluids, blood transfusion and dialysis if required.

Answer 40

``` respiratory syncytial virus (RSV) rhinoviruses parainfluenza influenza metapneumovirus adenovirus ```

Answer 41

``` streptococcus pneumoniae streptococci haemophilus influenzae bordetella pertussis mycoplasma pneumoniae ```

Answer 42

``` parental smoking overcrowded housing, damp housing poor nutrition underlying lung disease male gender haemodynamically significant congenital heart disease immunodeficiency ```

Answer 43

upper respiratory tract infection (common cold, sore throat (pharyngitis including tonsilitis), acute otitis media, sinusitis) Laryngeal tracheal infection (croup, bacterial tracheitis, acute epiglottitis) bronchiolitis pneumonia

Answer 44

``` acute onset rhinitis sore throat sneezing post-nasal drainage/drip cough fever non-specfic red pharynx nasal mucosal oedema ```

Answer 45

rhinoviruses, coronaviruses, RSV

Answer 46

pharynx and soft-palate are inflamed and local lymph nodes are enlarged and tender

Answer 47

pharyngitis tonsilitis laryngitis

Answer 48

intense inflammation of the tonsils often with a purulent exudate

Answer 49

Usually viral - most commonly caused by rhinovirus, coronavirus and adenovirus group A beta-haemolytic streptococci, and EBV

Answer 50

pain on swallowing fever >38 tonsillar exudate cervical lymphadenopathy

Answer 51

1st line - Phenoxymethylpenicillin | 2nd line - clarithromycin

Answer 52

the centor criteria - presence of tonsilar exudate - tender anterior cervical lymphadenopathy or lymphadenitis - history of fever - absence of cough 3 must be present

Answer 53

rapid antigen detection test | should be used routinely in patients with sore throat to allow immediate point-of-care assessment

Answer 54

it is a condition characterised by acute bronchiolar inflammation and a build up of mucus in the small airways of the lungs. usually caused by RSV virus - 75-80% of cases

Answer 55

it is almost exclusively an infantile disease - commonly affecting children under 2 most common cause of serious RTU in children under one maternal IgG provides protection to neonates higher incidence in winter months

Answer 56

rhinovirus influenza virus may be secondary to a bacterial infection

Answer 57

coryzal symptoms, including virus precede: Dry cough increasing breathlessness wheezing, fine inspiratory crackles feeding difficulties tachypnoea increased work of breathing may be present - retractions, grunting, nasal flaring

Answer 58

``` apnoea child looks seriously unwell to health care professional severe resp distress central cyanosis persistant oxygen sats less than 92% ```

Answer 59

a resp rate over 60BPM difficulty breast feeding or inadequate oral fluid intake clinical dehydration

Answer 60

largely clinical nasopharyngeal aspirate or throat swab - RSV rapid testing and viral cultures blood and urine cultures if child is pyrexic FBC ABG if severely unwell

Answer 61

supportive care, supplemental oxygen and mechanical ventiliation

Answer 62

monthly IM injection of palivizumab given to preterm babies born before 29 weeks, or babies born with chronic lung disease of prematurity before 32 weeks also can be given to those who are severely immunocompromised

Answer 63

Viral URTI | croup is also known as laryngotracheobronchitis - common respiratory disease of childhood

Answer 64

usually parainfluenza | other causes include RSV adenovirus, rhinovirus, enterovirus

Answer 65

commonly children who are between 6 months and 3 years | Autum

Answer 66

stridor (high pitched, wheezing sound caused by disrupted airflow) barking seal like cough, which is typically worse at night fever coryzal symptoms if severe there may be signs of resp distress

Answer 67

cyanosis lethargic/decreased level of consciousness labored breathing tachycardia

Answer 68

``` epiglottitis inhaled foreign body acute anaphylaxis bacterial tracheitis diptheria ```

Answer 69

aka bacterial tracheitis rare but very dangerous similar to viral croup but child has high fever, appears toxic and has rapidly progressive aiway obstruction with copious amounts of thick airway secretions it is caused by staphylococcus aureus treatment is with IV Abx, intubation and ventilation if needed

Answer 70

mild: Occasional barking cough No audible stridor at rest No or mild suprasternal and/or intercostal recession The child is happy and is prepared to eat, drink, and play moderate: Frequent barking cough Easily audible stridor at rest Suprasternal and sternal wall retraction at rest No or little distress or agitation The child can be placated and is interested in its surroundings severe: Frequent barking cough Prominent inspiratory (and occasionally, expiratory) stridor at rest Marked sternal wall retractions Significant distress and agitation, or lethargy or restlessness (a sign of hypoxaemia) Tachycardia occurs with more severe obstructive symptoms and hypoxaemia

Answer 71

moderate or severe croup < 6 months of age known upper airway abnormalities (e.g. Laryngomalacia, Down's syndrome) uncertainty about diagnosis (important differentials include acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation)

Answer 72

clinical diagnosis mild croup - single dose of oral dexamethasone moderate - single dose of oral dexamethasone plus nebulised adrenaline severe - single dose oral dexamethasone plus nebulized adrenaline plus oxygen

Answer 73

croup acute epiglotittis inhaled foreign body laryngomalacia

Answer 74

a chronic respiratory condition characterised by reversible and paroxysmal constriction of the airways secondary to type 1 hypersensitivity

Answer 75

``` personal or family history of atopy antenatal factors: maternal smoking, viral infection during pregnancy low birth weight not being breastfed maternal smoking around the child exposure to high concentrations of allergens (e.g. house dust mite) air pollution premature birth ```

Answer 76

IgE-mediated atopic conditions such as eczema alleric rhinitis (hay fever)

Answer 77

cold air atmospheric pollution NSAIDs and beta-blockers exposure to allergens Exercise

Answer 78

symptoms worse at night and early morning (diurnal variation) symptoms that have a non-viral trigger cough dyspnoea wheeze, chest tightness- episodic triggers increased work of breathing there may be expiratory wheeze on auscultation reduced peak expiratory flow rate

Answer 79

spirometry - FEV1 and FVC (FEV1 will be reduced, FVC normal ) peak expiratory flow response to bronchodilator on spirometry CXR ``` also consider skin prick testing fractional expired nitic oxide - elevated sputum culture exercise testing ```

Answer 80

``` bronchiolitis episodic viral wheeze inhaled foreign body recurrent aspiration cardiac failure CF primary ciliary dyskinesia ```

Answer 81

SABA when required add a regular preventer - very low dose ICS or LTRA if less than 5. Initial add on therapy - very low dose ICS plus inhaled LABA or LTRA next consider increasing ICS dose or add LTRA or LABA if no response to LABA consider stopping

Answer 82

viral episodic wheeze - wheezing only in response to viral infection and no interval symptoms. Usually resolves by 5 years. Triggered by viruses that can cause the common cold. Multiple trigger wheeze - wheeze in response to viral infection but also to another trigger such as exposure to aeroallergens and exercise. A significant proportion go on to have asthma

Answer 83

CF or bronchiectasis

Answer 84

treatment is symptomatic only first-line - SABA second line - LTRA - montelukast

Answer 85

trial of either ICS or LTRA - typically for 4-8 weeks

Answer 86

if after a high dose of bronchodilator therapy they: - have not responded adequately clinically - if they are becoming exhausted - have decreased oxygen sats less than 92% - if CXR is indicated - if there are unusual features or signs of severe infection

Answer 87

MILD: : SaO2 >92% in air, vocalizing without difficulty, mild chest wall recession and moderate tachypnoea MODERATE: SpO2 >92%, PEF>50% best or predicted, no clinical features of severe asthma SEVERE: SaO2 <92% PEFR 33-50%, cannot complete sentences in one breath or too breathless to feed or talk. Heart rate >125 (if over 5 years) or >140 (2-5 years) RR > 30 (over 5 years) or >40 (2-5 years) LIFE THREATENING: SaO2 <92% ,PEFR <33%, silent chest, poor resp effort, cyanosis, hypotension, exhaustion, confusion, normal C02

Answer 88

- give beta-2 agonist via a spacer - one puff every 30-60 seconds up to a max of 10 puffs if symptoms not controlled repeat and refer to hospital

Answer 89

if low oxygen sats give high flow oxygen SABA via nebuliser ipratropium bromide can be added if no response to SABA steroid therapy should be given for 3 days 2nd line IV salbutamol - essential to monitor for salbutamol toxicity magnesium sulphate can be considered

Answer 90

Newborn - organisms from the mother's genital tract particularly group B streptococcus, E.coli, klebsiella, staph aureus infants and young children - RSV most common, streptococcus pneumoniae, chlamydia trachomatis Children over 5 - mycoplasma pneumoniae, streptococcus pneumoniae and chlamydia pneumoniae ar the main causes at all ages mycobacterium tuberculosis should be considered

Answer 91

fever and difficulty breathing = the most common presenting symptoms usually preceded by an upper RTI cough lethary poor feeding unwell child localised chest, abdo or neck pain is a feature or pleural irritation and suggests bacterial infection examination will show signs of resp distress, desaturation and cyanosis tachypnoea, nasal flaring and chest indrawing end inspiratory coarse crackles over the affected area classical signs of consolidation - dull percussion, decreased breath sounds and bronchial breathing oxygen sats may be decreased

Answer 92

CXR - may confirm diagnosis but cannot differentiate between viral and bacterial Nasopharageal aspirate - viral immunoflourenence identify viral causes blood tests - FBC and acute phase reactants blood cultures

Answer 93

first line treatment is amoxicillin for all children with pneumonia for children under 5 alternatives include co-amoxiclav for tyical pneumonia (steptococcus oneumoniae) or clarithromycin for atypial pneumonia (mycoplasma pneumoniae and chlamydia trachomatis) if over 5 years - consider macrolide (clarithromycin) if mycoplasma or chlamydia is suspected, if staoh aureus suspected the consider macrolide or flucloxacillin with amoxicillin severe pneumonia: co-amoxiclav , cefotaxime or cefuroxime IV

Answer 94

most cases can be managed at home, admission is indicated if: - oxygen sats less than 92% - severe tachypnoea - difficulty breathing - grunting - apnoea - not feeding

Answer 95

an autosomal recesive disorder which leads to a defect in the CF transmembrane receptor protein which causes defective ion transport in exocrine glands. causes thickening of respiratory mucus - the lungs therfore prone to inadequate mucociliary clearance, chronic bacterial infections

Answer 96

all newborn babies are screened for CF | blood spot analysis on the Gurthrie card

Answer 97

infancy - meconium ileus, prolonged neonatal jaundice Childhood - recurrent lower respiratory chest infection, bronchiectasis, poor appetite, rectal prolapse, nasal polyps, sinusitis Adolescence: bronchiectasis, DM, cirrhosis and portal hypertension, distal intestinal obstruction, pneumothorax, haemoptyysis, male infetiltiy short stature delayed puberty female subfertility weight loss or poor weight gain 90% of children with CF have pancreatic exocrine insuficiency (lipase, amylase and proteases) resulting in maldigestion and malabsorption

Answer 98

sweat test - abnormally high sweat chloride immunoreactive trypsinogen test (new born screening) CXR glucose tolerance test LFT faecal elastase to assess pancreatic function genetic analysis

Answer 99

``` malnutrition adrenal insufficiency glycogen storage disease nephrogenic diabetes insupidus hypothyroidism G6PD ectodermal dysplasia ```

Answer 100

MDT approach annual review in specialist center For ongoing respiratory disease: twice daily physiotherapy to clear airway secretions and postural drainage, inhaled bronchodilator (salbutamol), inhaled mucolytic (dornase alpha), if they have chronic pseudomonas infection give inhaled tobramycin GI disease: monitoring and optimising nutrion pacreatic insuficiency - pancreatic enzyme replacement (pancreatin), H2 antagonist or PPI (ranitidine or omeprazole), fat soluble vitamin supplementation (A, D, K and E) liver disease - ursodeoxycholic acid

Answer 101

A cellulitis of the supraglottis it is a life threatening emergency due to high risk of respiratory obstruction caused by haemophilus influenzae type B (HiB) - Hib immunisation has led to a 99% reduction in the incidence most common in the age group 1-6 years but can affect all age groups **** important to distinguish between epiglottitis and croup

Answer 102

``` the onset is very acute/rapid high fever and generally unwell - toxic looking child stridor drooling of saliva increasing resp difficulty over hours sore throat tripod position ```

Answer 103

lateral neck radiograph (thumb print sign) FBC blood cultures Direct visualisation by senior (diagnosis)

Answer 104

secure airway and supplemental O2 IV Abx (cefotaxime or ceftriaxone or ampicillin or clindamycin) dexamethasone can be added to reduce inflammation inhaled adrenaline - if upper airways are compromised intubation once stable - oral Abx - amoxicillin or cefaclor

Answer 105

Epiglottitis is rapid onset (hours), croups is days croups has coryza prior to onset croup has barking cough, epiglottitis usually has no cough croup they can drink/feed epiglottitis will have drooling saliva epiglottitis will have a high fever croup has rasping stridor epiglottitis is a soft stridor in epiglottitis their voice will be weak or silent

Answer 106

Croup – harsh loud stridor (mostly viral, 6 months to 6 years of age, harsh loud stridor, coryza, mild fever and hoarse voice) Epiglottitis – (caused by H. influenzae type b, rare since Hub immunisation Bacterial tracheitis – harsh loud stridor, higher fever, toxic Inhaled foreign body – chocking on peanut, sudden onset of cough or respiratory distress Laryngomalacia – recurrent or continuous since birth.

Answer 107

AKA respiratory distress sydrome caused by inadequate production of surfactant type 2 from the pneumocytes in the lungs low surfactant leads to alveoli collapsing on expiration and this increases the energy needed for breathing

Answer 108

``` Premature maternal DM at term multiple pregnancy fam history of RDS csection hypothermia male>female ```

Answer 109

* Intrapartum asphyxia * RTI: GB-BHS pneumonia * Meconium aspiration pneumonia * Pulmonary haemorrhage * Pulmonary hypoplasia * Congenital diaphragmatic hernia

Answer 110

``` Early signs • Tachypnoea >60 • laboured breathing • grunting • recession: subcostal and intercostal • Nasal flaring • Cyanosis • Diminished breathing sounds Late signs • Fatigue • Apnoea • Hypoxia ```

Answer 111

1. surfactant replacement therapy: endotracheal tube 2. antibiotics 3. O2 : SaO2 85-93% • Mild: via a hood • Moderate: CPAP • Severe : endotracheal tube 4. Nutrition • If the infant is stable: IV nutrition: amino acids and lipids • If resp status is stable: small volume of gastic feed via a tube

Answer 112

bordetella pertussis

Answer 113

One week of coryza (Catarrhal phase), then the child develops a characteristic paroxysmal or spasmodic cough followed by a characteristic inspiratory whoop (paroxysmal phase). Often worse at night Often vomiting can occur In infants the whoop may be absent, but apnoea may occur at this age Epistaxis and subconjunctival haemorrhage may occur vigorous coughing Paroxysmal phase may last 3-6 weeks and symptoms gradually decrease but may persist for many months (convalescent phase)

Answer 114

nasal swab | PCR and serology

Answer 115

under 6 month - admit - notifiable disease - oral macrolide - clarithromycin - household contacts - prophylaxis - school exclusion for 48 hours following commencing antibiotics

Answer 116

subconjunctival haemorrhage pneumonia bronchiectasis seizures

Answer 117

face masks

Answer 118

adrenal suppression growth suppression osteoporosis high dose steroids may cause adrenal crisis

Answer 119

``` rhinitis otitis media pharyngitis tonsilitis larygitis Epiglottitis ```

Answer 120

``` bronchitis croup tracheitis bronchiolitis pneumonia ```

Answer 121

consolidation of the nasopharynx - -> upper airways mucosa infection - otitis media, sinusitis - ->lower airway mucosal infection - bacterial bronchitis, pneumonia - -> occult septicaemia, pneumonia with septicaemia, meningitis

Answer 122

resp disease characterised by inflammation of the lung parenchyma (excluding the bronchi) with congestion caused by viruses, bacteria or irritants

Answer 123

infection --> inflammation --> impaired muco-ciliary clearance --> infection

Answer 124

``` paediatric pulmonologist physio dietician nurse liason primary care team teacher psychologist ```

Answer 125

pneumonia, pulmonary oedema, bronchogenic cyst enlarged left atrium compressing left mainstem bronchus chest deformity asthma brochiolitis bronchitis CF polyps airway obstruction - foreign body, mucus, pus, blood

Answer 126

ventricular septal defect patent ductus arteriosus atrial septal defect they present as breathless

Answer 127

tetralogy of Fallot transposition of the great arteries they would present as blue

Answer 128

first breath = a fall in resistance to pulmonary blood flow - -> blood flow through the lungs increases six fold - ->this results in a rise in left atrial pressure. - -> mean while the blood returning to right atrium falls as the placenta is excluded from the circulation - -> the changes in the pressure difference causes the foramen ovale to close - the DA will normally close within the first few hours or days of life

Answer 129

symptoms - breathlessness (particularly on feeding or exertion), sweating, poor feeding, recurrent chest infection signs: poor weight gain or faltering growth, tachypnoea, tachycardia, heart murmur, gallop rhythm, enlarged heart, hepatomegaly, cold peripheries.

Answer 130

left heart obstruction e.g. coarctation of the aorta *if obstruction is very severe then arterial perfusion may be predominantly by right to left flow of the blood via the ductus arteriosus (duct dependant circulation) - closure of the duct = severe acidosis, collapse and death unless duct patency is restored

Answer 131

left to right shunt | this eventually causes pulmonary oedema and breathlessness due to decreased pulmonary vascular resistance

Answer 132

children will develop Eisenmenger syndrome which is irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary arterial pressure and flow. this lead the shunt reversing to a right to left shunt and results in a blue child. If this develops the only surgical option is a heart-lung transplant if available.

Answer 133

- cardiac disorders - cyanotic congenital heart disease - resp disorders - surfactant deficiency, meconium aspiration, pulmonary hypoplasia - persistent pulmonary hypertension of the new born - failure of the pulmonary vascular resistance to fall after birth - infection - septicaemia from group B streptococcus and other organisms - metabolic disease - metabolic acidosis and shock

Answer 134

secundum ASD partial/primum ASD

Answer 135

often asymptomatic may have recurrent chest infections from the fourth decade they may develop arrhythmias

Answer 136

an ejection systolic murmur best heard at the upper left sternal edge - due to increased flow across the pulmonary valve because of the left-to-right shunt a fixed and widely split second heart sound

Answer 137

CXR - cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular markings ECG - right bundle branch block Echo

Answer 138

Management options include: Active monitoring (small defects may close on their own) Percutaneous transvenous catheter closure (via the femoral vein) Open-heart surgery

Answer 139

smaller than the aortic valve in diameter - up to 3mm

Answer 140

often asymptomatic a loud pan-systolic murmur at lower left sternal edge (loud murmur = smaller defect) quiet pulmonary second sound CXR and ECG will be normal echo will demonstrate precise anatomy of defect management - often will close spontaneously

Answer 141

- bigger than the size of the aortic valve - symptoms of heart failure with breathlessness and FTT after 1 week old, recurrent chest infections, tachypnoea, tachycardia and enlarged liver from heart failure, active precordium. - there may be a soft or no murmur = large defect - there will be a loud pulmonary second sound from raised pulmonary pressure - CXR - cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema - ECG - biventricular hypertrophy by 2 months of age - ECHO - demonstrated anatomy of defect - Management: diuretic for heart failure (furosemide) often combined with captopril. Surgery should be performed at age 3-6 month

Answer 142

a form of congenital heart defect generally classed as 'acyanotic'. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis. connection between the pulmonary trunk and descending aorta usually the ductus arteriosus closes within the first 24 hrs of life due to increased pulmonary flow which enhances prostaglandins clearance more common in premature babies, born at high altitude or maternal rubella infection in the first trimester

Answer 143

``` left subclavicular thrill continuous 'machinery' murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat ```

Answer 144

1st line - IV indomethacin (inhibits prostaglandin synthesis ) - closes the duct in the majority of the cases *if it is associated with another congenital heart defect amenable to surgery then prostaglandin E1 is usefull to keep the duct open until after surgical repair use a percutaneous catheter device closure if baby too small or large duct then use surgical ligation

Answer 145

``` the hypoxia (nitrogen washout) test place them in 100% oxygen for 10 mins and then if right radial artery PaO2 from blood gas remains low then it Is cyanotic congenital heart disease ``` It works on the assumption that if there is right to left shunting in cyanotic heart disease, no amount of oxygenation in the pulmonary circulation will alter the desaturating effect of the shunt. However, if there is a pulmonary defect causing cyanosis this may be corrected by increasing the inspired oxygen.

Answer 146

- ventricular septal defect (VSD) - right ventricular hypertrophy - pulmonary stenosis (right ventricular outflow tract obstruction) - overriding aorta

Answer 147

cyanosis - hypercyanotic episodes ‘tet’ spells a loud harsh ejection systolic murmur in older children there may be clubbing may have tachypnoea

Answer 148

CXR - boot shaped heart ECG - will show right ventricular hypertrophy when older ECHO - will show the cardinal features.

Answer 149

complete surgical repair to close VSD and relieve RV outflow obstruction

Answer 150

the aorta is connected to the right ventricle and the pulmonary arteries are connected to the left ventricle - this means there is two parallel circulation *incompatible with life unless there is a mixing of blood - often there is naturally occurring VSD, ASD, PDA which can achieve mixing short term leads to right to left shunt

Answer 151

neonatal cyanosis usually appears on day 2 when ductal closure leads to reduced mixing of desaturated and saturated blood often a loud, single heart sound

Answer 152

maintain ductus arteriosus with prostaglandin infusion a balloon atrial septostomy all patients will require surgery, arterial switch procedure in the neonatal period

Answer 153

atrioventricular septal defect common in down-syndrome may be complete or partial it will present on antenatal USS screening cyanosis at birth or heart failure at 2-3 weeks of life manage heart failure and fix surgically at 3-6 months

Answer 154

Aortic stenosis is the most common valvular heart disease and the most common indication for valve replacement surgery. It refers to narrowing of the aortic valve, restricting blood flow from the left ventricle to the aorta.

Answer 155

Symptoms: chest pain, syncope/presyncope, dyspnoea Signs: slow rising pulse, narrow pulse pressure, ejection systolic murmur that radiates to the carotids, thrill

Answer 156

balloon dilatation

Answer 157

mainly asymptomatic small number of neonates have critical pulmonary stenosis and have duct- dependant circulation ejection systolic murmur best heard at the upper left sternal edge ejection click there may be a ventricular heave when stenosis is severe CXR - normal or post stenotic dilation of the pulmonary artey ECG - evidence of right ventricular hypertrophy

Answer 158

Coarctation of the aorta is a congenital heart defect in which your baby's aorta (the largest artery in their body) is pinched in or narrowed in one spot. This restricts normal blood flow to their lower body

Answer 159

``` often asymptomatic sometimes when the DA closes the aorta also constricts, causing severe obstruction of the LV outflow systemic hypertension ejection systolic murmur radiofemoral delay ```

Answer 160

CXR - rib notching due to the development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction, 3 sign - visible notch in the descending aorta at the site of the coarctation ECG- LV hypertrophy

Answer 161

insertion of a stent | sometimes surgical repair is required

Answer 162

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. usually VSD is present presentation is with shock in the neonatal period - circulatory collapse with absent femoral pulses and absent left brachial pulse

Answer 163

maintain ABC prostaglandin infusion complete correction with closure of VSD and repair of the aortic arch is usually performed within the first few days of life

Answer 164

underdevelopment of the left side of the heart small or atretic mitral valve left ventricle is diminutive and there is usually aortic valve atresia small ascending aorta circulatory collapse - all peripheral pulses absent sometimes detected antenatally no flow through the left side of the heart, so ductal constriction leads to profound acidosis and rapid CV collapse

Answer 165

it is normal in children and it detectable as a cyclical change in heart rate with respiration (acceleration during inspiration)

Answer 166

supraventricular tachycardia

Answer 167

rapid HR between 250-300BPM can cause poor CO and pulmonary oedema it will typically present as heart failure

Answer 168

narrow complex tachycardia (QRS less than 100ms)

Answer 169

``` circulatory and respiratory support vagal stimulation manoeuvres e.g. carotid sinus massage IV adenosine electrical cardioversion flecainide ```

Answer 170

rare condition usually related to the presence of anti-RO or anit-LA antibodies in maternal serum - this mothers will either manifest or latent connective tissue disorders the antiboides appear to prevent the normal development of the conduction system in the developing heart with atrophy and fibrosis of the AV node all children with symptoms will require the insertion of an endocardial pacemaker

Answer 171

associated with sudden loss of consciousness during exercise, stress or emotion usually in late childhood may be mistakenly diagnosed as epilepsy inheritance is AD

Answer 172

there is an abnormal immune response to a streptococcal infection usually develops 2-4 weeks following the infection it is usually a pharyngeal infection - then polyarthritis, mild fever and malaise develop

Answer 173

raised ASO titre or other streptococcal antibodies positive throat swabs positive rapid group A strep antigen test

Answer 174

Two major or one major and two minor MAJOR: erythema marginatum - uncommon early manifestation rash on trunk and limbs, Sydenham's chorea - a late feature - involuntary movements and emotional liability, polyarthritis, pancarditis (endocarditis, myocarditis, pericarditis), subcutaneous nodules MINOR: fever, raised ESR or CRP, arthralgia, prolonged PR interval

Answer 175

the most common long-term damage is mitral stenosis from scarring and fibrosis.

Answer 176

Outline of management antibiotics: oral penicillin V anti-inflammatories: NSAIDs are first-line treatment of any complications that develop e.g. heart failure recurrence should be prevented with monthly injections of benzathine penicillin

Answer 177

congenital heart disease rheumatic fever valve disease prosthetic valves IVDU

Answer 178

it should be suspected in any child or adult with fever, malaise, raised ESR, unexplained anaemia or haematuria

Answer 179

``` streptococcus viridans - linked to poor dental hygiene staphylococcus aureus (common in IVDU) ```

Answer 180

blood cultures should be taken before starting Abx detailed cross-section echo may confirm diagnosis but will not exclude diagnosis acute phase reactants are raised

Answer 181

high dose IV Abx -Amoxicillin plus gentamicin or vancomycin for a min of 4-6 weeks

Answer 182

``` large left to right shunt left sided obstructive lesion cardiomyopathy myocarditis endocarditis Kawasaki disease tachyarrhythmias ```

Answer 183

``` sweating breathless, tachypnoea, coughing, lung creps poor feeding, poor weight gain and FTT hepatomegaly cardiomegaly tachycardia, gallop heart rhythm ```

Answer 184

``` VSD ASD PDA pulmonary valve disease coarctation of the aorta aortic stenosis hypoplastic left heart syndrome hypertrophic obstructive cardiomyopathy dextrocardia ```

Answer 185

tetralogy of fallot transposition of the great arteries tricuspid atresia

Answer 186

- Infections (coxsackie B , EBV, strept, TB, toxoplasmosis - connective tissue (RA, rheumatic fever, SLE, sarcoidosis - metabolic - hyperuricaemia, hypothyroidism - malignancy - radiotherapy

Answer 187

typically sharp pain which is exacerbated and exaggerated by lying down and relieved by sitting or leaning forward. Pain is often referred to left shoulder

Answer 188

analgesia anti-inflammatory drugs (colchicine, aspirin, ibuprofen) pericardiocentesis for pericardial effusion

Answer 189

infections Kawasaki drugs - Adriamycin connective tissue disease: SLE, RA, rheumatic fever, sarcoidosis

Answer 190

hypertrophic dilated restrictive

Answer 191

inherited secondary to metabolic disease direct result from a viral infection

Answer 192

gastro-oesophageal reflux disease - when there is the inappropriate effortless passage of gastric contents into the oesophagus

Answer 193

it is associated with slow gastric emptying, liquid diet, horizontal position, and low resting lower oesophageal sphincter pressure. ``` lower oesophageal sphincter dysfunction (hiatus hernia) may cause reflux increased gastric pressure external gastric pressure gastric hypersecretion of acid food allergy cerebral palsy ```

Answer 194

common in the first 5 years of life but usually all symptoms clear by 12 months.

Answer 195

typically develops before 8 weeks vomiting/regurgitation milky vomits after feeds may occur after being laid flat excessive crying, especially while feeding

Answer 196

``` oesophageal stricture barrett's oesophagus (squamous to columnar epithelium) faltering growth anaemia lower resp disease ```

Answer 197

usually diagnosed clinically nurse infants on a head up slope of 30 degrees thicken milk feeds in infants, small frequent meals, avoid food before sleep, avoid fatty foods, cirtus juices, caffeine and fizzy drinks add drugs if severe - ranitidine or omeprazole

Answer 198

idiopathic hypertrophic pyloric stenosis | hypertrophy of the pylorus muscle causing outlet obstruction

Answer 199

usually in the third or fourth week of life | ** it is more common in boys

Answer 200

projectile vomiting, non-bilious, may contain altered blood (coffee ground) or fresh blood from oesophagitis. Vomiting will occur within an hour of feeding and the baby is immediately hungry. If they present early (2nd/3rd week) then vomiting may not be projectile. weight loss constipation dehydration, malnutrition and jaundice are late sings palpable mass in the upper abdomen

Answer 201

test feed - peristalsis seen during feed, the pyloric tumour is usually easiest felt early in the feed or after the baby has vomited US - if tumour cannot be felt, USS will confirm or exclude the diagnosis biochemistry - hypochloraemic, hypokalaemic metabolic alkalosis

Answer 202

rehydrate and correct the alkalosis before surgery IV fluids withold feeds - the stomach should be emptied with an NGT Ramstedt's pyloromyotomy

Answer 203

it is a type of inflammatory bowel disease that may affect any part of the GI tract but terminal ilium and proximal colon are commonest sites of involvement bowel involvement is non-continuous (skip lesions)

Answer 204

``` abdominal pain diarrhoea +/- blood/mucus weight loss lethary fever oral lesions perianal skin tag uveitis erythema nodosum ```

Answer 205

FBC - anaemia may be present, leukocytosis is associated with acute or chronic inflammation, thrombocytosis is a useful marker for active inflammation iron studies, B12, and folate there may be hypoalbuminaemia, hypocholesterolaemia, and hypocalcaemia raised ESR and CRP stool sample - to rule out and infectious cause (faecal calprotectin- inflam) endoscopy CT or MRI - helps to localise disease

Answer 206

Glucocorticoids (prednisolone) are generally used to induce remission. Budesonide is an alternative. 5-ASA (mesalazine) are used second line but are not as effective. Azathioprine and mercaptopurine may be used as add on therapy. Methotrexate is an alternative azatioprine. infliximab useful in refractory disease or fistulating disease. Azathioprine or mercaptopurine are used first line to maintain remission. surgical management: local surgical resection for severe localised disease e.g. strictures, fistula, may be indicated

Answer 207

a form of inflammatory bowel disease, inflammation always starts at the rectum and never spreads beyond the ileocaecal valve and is continuous.

Answer 208

``` bloody diarrhoea urgency tenesmus abdominal pain, particularly in the lower left quadrant weight loss growth failure ``` in children it is usually pancolitis where as in adults it is usually confined to the distal colon

Answer 209

amino salicylates are used to induce remession (mesalazine) oral corticosteroid can also be used more agressive disease may need immunomodulator therapy (azathioprine) to maintain remission if severe - manage in hospital *methotrexate is not recommended in UC

Answer 210

``` colonoscopy biopsies stool studies FBC ESR CRP barium enema - will show loss of haustrations, superficial ulceration and in long-standing disease - the colon is narrow and short ```

Answer 211

rotavirus (most common) caliciviruses (norovirus and sapovirus) adenovirus astrovirus

Answer 212

``` vomiting non-bloody diarrhoea cramping abdominal pain low grade fever dehydration ```

Answer 213

- infants under the age of 6 months or those born with a low birth weight - if they have passed more than 6 diarrhoeal stools in the previous 24 hours - if they have vomited three times of more in the past 24 hours - if they have been unable to tolerate/not been offered extra fluids - if they have malnutrition

Answer 214

mild: (less than 5% loss of body weight): skin turgor may be dcreased, dry mucous membranes, low urine output, HR increased, BP normal, perfusion normal, pale, may be irritable moderate: (5-10% loss of body weight): skin turgor decreased, very dry mucous membranes, oliguric, HR increased, BP may be normal, prolonged cap refil, grey skin colour, lethargic severe: (10-15% loss of body weight): poor skin turgor with tenting, parched mucous membranes, anuric, increased HR, BP decreased, prolonged cap refil, mottled skin, comatose

Answer 215

appears unwell or deteriorating, altered responsiveness, tachycardia, tachypnoea, skin turgor reduced

Answer 216

Isonatraemic - losses of sodium and water are proportional and the plasma sodium levels remains within a normal range hyponatraemic - if the child with diarrohea drinks large quantities of water, it leads to a fall in plasma sodium - this leads to a shift of water from extra cellular to intracellular compartments which increases cellular volume - may result in convulsions

Answer 217

water losses exceeds the relative sodium loss and the plasma sodium concentration increases the extracellular fluid becomes hypertonic with respect to intracellular fluid and this leads to a shift of water into the extracellular space from the intracellular compartments this form of dehydration is hard to see as there is not signs of extracellular fluid delpetion. This type of dehydration occurs when there is high insensible water loss (when water is lost through the skin - high fever or hot dry enviroment) or from perfuse, low sodium diarrhoea. water is drawn out of the brain and cerebral shrinkage within rigid skull may lead to jittery movement, increased muscle tone with hyperreflexia, altered consciousness, seizures and multiple small cerebral haemorrhages

Answer 218

fluids - oral rehydration solution mild dehydration - less than 5% loss of body weight give 50mls/kg plus maintenance fluids moderate (less than 10%) - 100ml/kg plus maintenece if shock add fluid bolus

Answer 219

it is most common in children under 2 ``` salmonella campylobacter jejuni shigella E. coli clostridium difficile Bacillus cereus ``` sources of infection include contaminated water, poor food hygiene, faecal-oral route

Answer 220

``` watery diarrhoea vomiting cramping abdo pain fever dehydration electrolyte disturbance malaise blood and mucus in diarrhoea abdo pain may mimic appendicitis or IBD tenesmus ```

Answer 221

``` bacteraemia secondary infections - pneumonia, osteomyelitis, meningitis Reiter's syndrome GBS haemolytic-uraemic syndrome reactive arthropathy haemorrhagic colitis ```

Answer 222

stool +/- culture stool clostridium difficile toxin sigmoidoscopy

Answer 223

anorexia abdominal pain - initially central but localises to the RIF low-grade fever nausea diminished bowel signs *younger children or children with a retrocaecal/pelvic appendix may present atypically it is uncommon in children under the age of 4 - however if these children do get it, it often presents when perforated

Answer 224

FBC - mild neutrophil predominant leukocytosis (increase in WCC) pelvic or abdominal CT USS urinalysis - to rule out other causes

Answer 225

appendectomy plus supportive care | IV antibiotics - cefoxitin

Answer 226

it is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm - this can result in pulmonary hypoplasia and hypertension which can cause respiratory distress shortly after birth

Answer 227

left-sided posterolateral Bochdalek hernia (85%) | - there is left sided herniation of abdominal contents through the posterolateral foramen of the diaphragm

Answer 228

the apex beat and the heart sounds will be displaced to the right side of the chest wall, with poor air entry to the left side

Answer 229

usually diagnosed on antenatal USS, but the prognosis for the foetus is poor if the diagnosis is not made antenataly and the baby present at birth, clinical finding may include resp distress, scaphoid abdomen and apparent dextrocardia. diagnosis can be confirmed with x-ray

Answer 230

initial management: sedation, paralysis, endotracheal intubation and mechanical ventilation with 100% oxygen. NGT placement avoid bag mask valve ventilation after stabilisation the hernia is repaired surgically - however often the more pressing problem is lung hypoplasia - where the pressing of the herniated viscera has prevented the lungs developing - lung hypoplasia =high mortality *only around 50% of newborn survive

Answer 231

herniation of the stomach into the chest wall through the oesophageal hiatus in the diaphragm sliding rolling

Answer 232

radiologically by barium swallow test/barium meal

Answer 233

a reducible swelling in the groin, often extending to the scrotum more common in boys and preterm babies most commonly on the right side due to later descent of the right testis 15% are bilateral symptoms are rare

Answer 234

reduce following opioid analgesia and sustained compression. Surgery is delayed for 24-48 hours to allow resolution of oedema. If reduction is impossible, emergency surgery is required because of the risk of strangulation of bowel and damage to the testis

Answer 235

infrequent passage of stool associated with pain and difficulty or delay in defaecation

Answer 236

children less than 1: - fewer than 3 complete stools per week (type 3 or 4 on bristol stool chart - rabbit dropping or hard large stools) - this does not apply for children who are exclusively breastfed as these infants may not pass stools for several days - distress on passing stools, bleeding associated with stool straining children older than 1: - fewer than 3 complete stools per week, overflowing soiling, rabbit dropping, large infrequent hard stools that can block the toilet - poor appetite that improves with the passage of a large stool, waxing and wining of abdominal pain with passage of stool, straining, anal pain

Answer 237

``` often idiopathic other causes: low fibre diet lack of mobility and exercise poor colonic motility medications - opiates and anticholinergics anal fissure Hirschsprung's disease hypothyroidism hypercalcaemia sexual abuse UTI ```

Answer 238

``` difficult or painful defecation long interval between passing stools faecal incontinence overflow faecal incontinence palpable faecal mass in abdomen otherwise healthy child abdominal distension abdominal pain ```

Answer 239

- failure to pass meconium within 24 hours of life - indicates hirschsprung disease - failure to thrive/grow - indicates hypothyroidism or coeliac disease - gross abdominal distension -> indicated hirschprung disease or other gastrointestinal dysmotility - abnormal lower limb neurology deformity -> lumbosacral pathology - sacral dimple over the natal cleft, over the spider naevus, hairy patch, central pit or discoloured skin -> spina bifida - abdnormality of appearance or postion of anus --> abnormal anatomy - bruising -> sexual abuse - fistulae, abscesses or fissures -> perianal crohn disease

Answer 240

dietary modification (increase fluids and fibre) start with a osmotic laxative (lactulose/ movicol) add a stimulant laxative (senna) if they are over one year of age a stool softener can be added - macrogol laxitive - polyethylene glycol lus electrolytes * if they are under 4 years old had have faecal impactation a stimulant laxative should not be used occasionally retention is so severe that evacuation is only possible using enemas or by manual evacuate under an anaesthetic

Answer 241

the absence of ganglionic cells from the myenteric plexus (Auerbach’s plexus) and submucosal plexus of part of the large bowel results in a narrow, contracted segment. The abnormal bowel extends from the rectum for a variable distance proximally, ending in a normally innervated dilated colon. in most cases it is confined to the rectum or sigmoid - short segment disease however sometimes it involves the entire colon - long segment disease

Answer 242

usually withing the first few days of life with intestinal obstruciton - failure to pass meconium in the first 24 hours later abdominal distension and later bile stained vomiting develop * sometimes children with short segment disease will represent later on in childhood with chronic constipation.

Answer 243

abdominal x-ray will show intestinal obstrucion anorectal manometry or barium studies may be useful in giving the surgeon an idea of the length of the aganglionic segment but are unreliable for diagnostic purposes rectal biopsy will show no ganglionic cells in the submucosa

Answer 244

male and down syndrome

Answer 245

Initially, bowel irrigation/rectal washouts management is surgically, initially colostomy followed by anastomosing normally innervated bowel to the anus (pull-through procedure)

Answer 246

hirschsprung's enterocolitis - a dramatic gastroenteric illness characterised my abdominal distension, bloody watery diarrhoea, circulatory collapse and septicaemia. usually associated with clostridium difficile toxin in the stools - mortality is 10%

Answer 247

autoimmune disease triggered by dietary gluten peptides | is an enteropathy due to lifelong intolerance to gluten

Answer 248

the gliadin fraction of gluten provokes a damaging response in the proximal small intestine mucosa

Answer 249

``` a positive family history type 1 diabetes down syndrome IgA deficiency autoimmune thyroid disease ```

Answer 250

the classical initial features include: - pallor - diarrhoea - pale, bulky floating stools - anorexia - failure to thrive - irritibilty later there is apathy, gross motor developmental delay, ascites, peripheral oedema, anaemia, delayed puberty, arthralgia, hypotonia, muscle waisting, specific nutrtional disorders

Answer 251

often is it diagnosed on screening of children at increased risk diagnosis suggested by positive serology, conformation depends upon the demonstration of mucosal changes - jejunal biopsy showing subtotal villous atrophy, increased intraepithelial lymphocytes, crypt hypertrophy -anti-endomysial IgA antibodies - tissue transglutaminase IgA antibody - anti-gliadin antibodies

Answer 252

gluten free diet | calcium and vitamin D deficiency

Answer 253

rehydration correction of electrolyte abnormalities corticosteroids

Answer 254

suboptimal weight gain in infants and toddlers growth falls away from standardized wight or height centile weight is the most sensitive indicator in infants and young children, height is the best indicator in older children *under stress - head circumfrance growth is more preserved than linear growth

Answer 255

mild - falls across two centile lines | severe - falls across three centile lines

Answer 256

birth weight reflects intrauterine enviroment - it is a poor guid to the childs correct genetic potential and weight may naturally fall until the correct level is attained.

Answer 257

usually classified as organic or non-organic organic include: impaired suck/swallow - oro-motor dysfunction, neurological disorder e.g. cerebral palsy, chronic ilness, malabsorption, inadequate retention - vomiting or severe GORD, failure to utilise nutrients , increased requirements - thyrotoxicosis non-organic - broad spectrum of psychosocial and enviromental deprivation - neglect, socioeconomic deprivation, mothers poor mental health

Answer 258

severe macronutrient malnutrition

Answer 259

severe protein malnutrition where there is generalised oedma as well as wasting

Answer 260

weight for age (a measurement of wasting and an index of acute malnutrition) mid upper arm circumference height for age - a measurment of stunting and an index of chronic malnutrition

Answer 261

bilateral pitting oedema a flacky-paint skin rash with hyperkeratosis (thickened skin) and desquamation distended abdomen and enlarged liver angular somatitis hair which is sparse and depigmented diarrhoea, hypothermia, bradycardia and hypotension low plasma albumin, potassium, glucose and magnesium

Answer 262

uncomplicated: community based therapy with ready to use therapeutic food and also a broad spectrum antibiotic - amoxicillin complicated: empirical antibiotic therapy (gentamicin and ampicillin) vitiman A supplementation shock - oxygen and fluids dehydration - rehydration solution for malnutrition (ReSoMal) hypoglycaemia - supplemental glucose or sucrose correct electrolyte imbalance hypothermia - gentle waming - especially at night

Answer 263

common cause of intestinal obstruction in young children and is defined as the prolapse of one part of the intestine into the lumen of the adjoining distal part.

Answer 264

the ileocaecal region | most commonly occuring in infants aged between 3 and 12 months

Answer 265

paroxysmal severe colicky pain and pallor, during episodes the child becomes pale they initially recover between painful periods but subsequently become increasingly lethargic Inconsolable crying During paroxysm infant will characteristically draw knees up vomiting red current jelly stool (late sign) Sausage-shaped mass in RUQ

Answer 266

x ray USS - will show target sign/doughnut sign diagnostic enema

Answer 267

fluid resuscitation unless there are signs of peritonitis, contrast enema reduction should be tried first antibiotics should be given - clindamycin and gentamicin if fails or contraindicated surgical reduction will be required

Answer 268

Meckel's diverticulum is the most common congenital anomaly of the gastrointestinal tract. It results from incomplete obliteration of the vitelline duct leading to the formation of a true diverticulum of the small intestine contains ectopic ileal, gastric or pancreatic mucosa occurs in 2% of the population is 2 feet from the ileocaecal valve is 2 inches long

Answer 269

usually asymptomatic abdominal pain which may mimic appendicitis rectal bleeding intestinal obstruction

Answer 270

FBC technetium scan - most useful to diagnose meckel's diverticulum USS abdominal Xray

Answer 271

only treat if symptomatic | excision of the diverticulum

Answer 272

chronic non-specific diarrhoea that occurs from 6 months to five years stools vary in consistency and often contain undigested food affected children are often well and thriving and there are no precipitating dietary factors

Answer 273

reassurance, dietary (increase fat intake, normalise fibre intake, reduce milk, fruit juice and sugary drink intake), loperamide (Imodium) may sometimes be needed.

Answer 274

a common set of benign symptoms seen in young children typically occurs in infants less than three months characterised by excessive cry and pulling up of the legs which is often worse in the evening often accompanied by excessive flatus occurs in upto 40% of infants and usually the cause is unknown if it is persistant then it may be due to cow's milk protein allergy or gasto-oesophageal reflux

Answer 275

is a progressive idiopathic, necroinflammatory process that may involve a segment or the entire biliary tree. there is destruction or absence of the extrahepatic biliary tree and intrahepatic biliary ducts

Answer 276

neonatal jaundice babies have a normal birth weight but fail to thrive as disease progresses pale stools and dark urine bruising - due to vitamin k deficiency related to chronic cholestasis hepatomegaly - not until disease has progressed, splenomegaly will often develop secondary to portal hypertension there is obstruciton of the bile ducts which causes bile to go to the liver and cause increase of conjugated bilirubin in the blood

Answer 277

serum total and conjugated bilirubin (high) prothrombin time, INR FBC - in advanced disease - low platelets and low WBC LFTs abdominal USS - will demonstrate contracted or absent gallbladder liver biopsy will show extrahepatic biliary obstruction - although features may overlap with neonatal hepatitis

Answer 278

kasai procedure AKA hepatoportoenterostomy - although even when successful can progress to cirrhosis and portal hypertension

Answer 279

an autosomal recessive disorder caused by a defect in the ATP7B gene located on chromosome 14 reduced synthesis of ceruloplasmin (the copper binding protein) and defective excretion of copper in the bile, which leads to an accumulation of copper in the liver, brain, kidney and cornea.

Answer 280

- Kayser Fleischer rings - blue nails - Hepatitis/liver cirrhosis - Neuro and psych (basal ganglia degeneration)

Answer 281

low serum ceruloplasmin LFTs 24 hour urine copper liver biopsy - elevated liver copper Genetic testing

Answer 282

with penicillamine or trientine - both promote urinary copper excretion zinc - to reduce copper absorption can be fatal from hepatic complications if left untreated

Answer 283

cystic dilations of the extrahepatic biliary system

Answer 284

25% present in infancy with cholestasis, in the older age group they present with abdominal pain, a palpable mass and jaundice or cholangitis

Answer 285

diagnosis with USS or radionuclide scanning | treatment is with surgical excision of the cyst

Answer 286

- Tachypnoea >60 - Grunting - Pale/mottled/blue - Looks ill to a healthcare professional - Weak, high-pitched or continuous cry - Age <3 and temp >38 - Non-blanching rash - Focal seizures - Neck stiffness - Moderate or severe chest indrawing

Paeds 1 🫁 🫀🚽 Flashcards

Renal, Resp, Cardo, GI