Paediatrics Flashcards

Question

Congenital heart disease

Answer 1

Acyanotic - most common causes -ventricular septal defects (VSD) - most common, accounts for 30% -atrial septal defect (ASD) -patent ductus arteriosus (PDA) -coarctation of the aorta -aortic valve stenosis VSDs are more common than ASDs. However, in adult patients ASDs are the more common new diagnosis as they generally presents later. Cyanotic - most common causes (3T's) -tetralogy of Fallot -transposition of the great arteries (TGA) -tricuspid atresia Fallot's is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot's generally presenting at around 1-2 months The presence of cyanosis in pulmonary valve stenosis depends very much on the severity and any other coexistent defects.

Answer 2

Pass medicine

Answer 3

The law around consent in children is complicated. However, as a rough guide: -patients less than 16 years old may consent to treatment if they are deemed to be competent (an example is the Fraser guidelines, previously termed Gillick competence), but cannot refuse treatment which may be deemed in their best interest -between the ages of 16-18 years it is presumed patients are competent to give consent to treatment -patients 18 years or older may consent to treatment or refuse treatment With regards to the provision of contraceptives to patients under 16 years of age the Fraser Guidelines state that all the following requirements should be fulfilled: -the young person understands the professional's advice -the young person cannot be persuaded to inform their parents -the young person is likely to begin, or to continue having, sexual intercourse with or without contraceptive treatment -unless the young person receives contraceptive treatment, their physical or mental health, or both, are likely to suffer -the young person's best interests require them to receive contraceptive advice or treatment with or without parental consent

Answer 4

Management; Non-pharmacological- diet (fluid and fibre, reduce cows' milk), behavioural training (encourage regular toileting, bowel diaries and reward systems), education: cause and involuntary nature of soiling Pharmacological- Disimpaction (movicol- may get increased soiling initially, then senna if that doesn't work), then maintenance dose (movicol) Complications; -faecal impaction -bedwetting (always ask about stools in an enuresis history) -anal fissure -rectal prolapse

Answer 5

Both immediate (IgE mediated) and delayed (non-IgE mediated) reactions are seen. The term CMPA is usually used for immediate reactions and CMPI for mild-moderate delayed reactions. Features -regurgitation and vomiting -diarrhoea -urticaria, atopic eczema -'colic' symptoms: irritability, crying -wheeze, chronic cough -rarely angioedema and anaphylaxis may occur Diagnosis is often clinical (e.g. improvement with cow's milk protein elimination). Investigations include: -skin prick/patch testing -total IgE and specific IgE (RAST) for cow's milk protein If the symptoms are severe (e.g. failure to thrive) refer to a paediatrician. Management if formula-fed; -extensive hydrolysed formula (eHF) milk is the first-line replacement formula for infants with mild-moderate symptoms amino acid-based formula (AAF) in infants with severe CMPA or if no response to eHF Management if breastfed; -continue breastfeeding -eliminate cow's milk protein from maternal diet. -use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months

Answer 6

a form of upper respiratory tract infection seen in infants and toddlers. It is characterised by stridor which is caused by a combination of laryngeal oedema and secretions. Parainfluenza viruses account for the majority of cases. (bronchiolitis is lower, croup and epiglottis are upper) Epidemiology -peak incidence at 6 months - 3 years -more common in autumn Features -stridor -barking cough (worse at night) -fever -coryzal symptoms CKS suggest admitting any child with moderate or severe croup. Other features which should prompt admission include: -< 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) Investigations -diagnosed clinically however, if a chest x-ray is done: -a posterior-anterior view will show subglottic narrowing, commonly called the 'steeple sign' Management -a single dose of oral dexamethasone (0.15mg/kg) to all children regardless of severity (check max dose, and uses BNF children) -prednisolone is an alternative if dexamethasone is not available Emergency treatment -high-flow oxygen -nebulised adrenaline (for severe croup)

Answer 7

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 8

The nitrogen washout test (also known as the hyperoxia test) may be used to differentiate cardiac from non-cardiac causes. The infant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease Initial management of suspected cyanotic congenital heart disease -supportive care -prostaglandin E1 e.g. alprostadil (used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect, this can act as a holding measure until a definite diagnosis is made and surgical correction performed) NB- Acrocyanosis is often seen in healthy newborns and refers to the peripheral cyanosis around the mouth and the extremities (hands and feet)

Answer 9

An autosomal recessive disorder causing increased viscosity of secretions (e.g. lungs and pancreas). It is due to a defect in the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a cAMP-regulated chloride channel Presenting features -neonatal period (around 20%): meconium ileus, less commonly prolonged jaundice -recurrent chest infections (40%) -malabsorption (30%): steatorrhoea, failure to thrive -other features (10%): liver disease Many patients are picked up during newborn screening programmes (raised IRT) or early childhood, but some are picked up after 18 years. If IRT is raised, then they undergo a sweat test (raised in CF) Other features of cystic fibrosis -short stature -diabetes mellitus -delayed puberty -rectal prolapse (due to bulky stools) -nasal polyps -male infertility, female subfertility

Answer 10

-Lifestyle- regular (at least twice daily) chest physiotherapy and postural drainage. Deep breathing exercises, high calorie diet, including high fat intake NB- minimise contact with each other CF patients to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa -Medical- vitamin supplementation, pancreatic enzyme supplements, lumacaftor (patients who are homozygous for the delta F508 mutation), long term prophylactic flucloxacillin to prevent staph aureus infection -Surgery- lung transplantation (chronic infection with Burkholderia cepacia is an important CF-specific contraindication to lung transplantation)

Answer 11

Referral points -doesn't smile at 10 weeks -cannot sit unsupported at 12 months -cannot walk at 18 months Fine motor skill problems -hand preference before 12 months is abnormal and may indicate cerebral palsy Gross motor problems -most common causes of problems: variant of normal, cerebral palsy and neuromuscular disorders (e.g. Duchenne muscular dystrophy) Speech and language problems -always check hearing -other causes include environmental deprivation and general development delay

Answer 12

Risk factors -female sex: 6 times greater risk -breech presentation -positive family history -firstborn children -oligohydramnios -birth weight > 5 kg Screening for DDH the following infants require a routine ultrasound examination -first-degree family history of hip problems in early life -breech presentation at or after 36 weeks gestation, irrespective of presentation at birth or mode of delivery -multiple pregnancy NB- all infants are screened at both the newborn check and also the six-week baby check using the Barlow and Ortolani tests (and USS) Clinical examination -Barlow test: attempts to dislocate an articulated femoral head -Ortolani test: attempts to relocate a dislocated femoral head other important factors include: -symmetry of leg length -level of knees when hips and knees are bilaterally flexed -restricted abduction of the hip in flexion Imaging -ultrasound is generally used to confirm the diagnosis if clinically suspected -however, if the infant is > 4.5 months then x-ray is the first line investigation Management -most unstable hips will spontaneously stabilise by 3-6 weeks of age -Pavlik harness (dynamic flexion-abduction orthosis) in children younger than 4-5 months -older children may require surgery

Answer 13

not done (pass medicine)

Answer 14

Gastroenteritis -main risk is severe dehydration -most common cause is rotavirus - typically accompanied by fever and vomiting for the first 2 days. The diarrhoea may last up to a week -treatment is rehydration Chronic diarrhoea -most common cause in the developed world is cows' milk intolerance -toddler diarrhoea: stools vary in consistency, often contain undigested food -coeliac disease -post-gastroenteritis lactose intolerance

Answer 15

Pass medicine (print)

Answer 16

Clinical features; face: upslanting palpebral fissures, epicanthic folds, Brushfield spots in iris, protruding tongue, small low-set ears, round/flat face flat occiput single palmar crease, pronounced 'sandal gap' between big and first toe hypotonia congenital heart defects (40-50%, see below) duodenal atresia Hirschsprung's disease Cardiac complications multiple cardiac problems may be present endocardial cushion defect (most common, 40%, also known as atrioventricular septal canal defects) ventricular septal defect (c. 30%) secundum atrial septal defect (c. 10%) tetralogy of Fallot (c. 5%) isolated patent ductus arteriosus (c. 5%) Later complications subfertility: males are almost always infertile due to impaired spermatogenesis. Females are usually subfertile, and have an increased incidence of problems with pregnancy and labour learning difficulties short stature repeated respiratory infections (+hearing impairment from glue ear) acute lymphoblastic leukaemia hypothyroidism Alzheimer's disease atlantoaxial instability

Answer 17

an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function. Features; -progressive proximal muscle weakness from 5 years -calf pseudohypertrophy -Gower's sign: child uses arms to stand up from a squatted position -30% of patients have intellectual impairment Investigation; -raised creatinine kinase -genetic testing has now replaced muscle biopsy as the way to obtain a definitive diagnosis Management -is largely supportive as unfortunately there is currently no effective treatment Prognosis -most children cannot walk by the age of 12 years -patients typically survive to around the age of 25-30 years -associated with dilated cardiomyopathy

Answer 18

Ebstein's anomaly is a congenital heart defect characterised by low insertion of the tricuspid valve resulting in a large atrium and small ventricle. It is sometimes referred to as 'atrialisation' of the right ventricle. Ebstein's anomaly may be caused by exposure to lithium in-utero. Associations -patent foramen ovale (PFO) or atrial septal defect (ASD) is seen in at least 80% of patients, resulting in a shunt between the right and left atria -Wolff-Parkinson White syndrome Clinical features -cyanosis -prominent 'a' wave in the distended jugular venous pulse, -hepatomegaly -tricuspid regurgitation (pansystolic murmur, worse on inspiration) -right bundle branch block → widely split S1 and S2

Answer 19

-if a topical steroid is also being used the emollient should be applied first followed by waiting at least 30 minutes before applying the topical steroid -creams soak into the skin faster than ointments -emollients can become contaminated with bacteria - fingers should not be inserted into pots (many brands have pump dispensers, or you can use a spatula/spoon)

Answer 20

A congenital cyst found in the mouth. They are common on the hard palate, but may also be seen on the gums where the parents may mistake it for an erupting tooth. No treatment is generally required as they tend to spontaneously resolve over the course of a few weeks.

Answer 21

Management following a seizure -children who have had a first seizure OR any features of a complex seizure should be admitted to paediatrics Prognosis -the overall risk of further febrile convulsion = 1 in 3. - -if recurrences, try teaching parents how to use rectal diazepam or buccal midazolam. Parents should be advised to phone for an ambulance if the seizure lasts > 5 minutes -regular antipyretics have not been shown to reduce the chance of a febrile seizure occurring Link to epilepsy -risk factors for developing epilepsy include a family history of epilepsy, having complex febrile seizures and a background of neurodevelopmental disorder -children with no risk factors have 2.5% risk of developing epilepsy -if children have all 3 features the risk of developing epilepsy is much higher (e.g. 50%)

Answer 22

Baby may show symptoms of alcohol withdrawal at birth e.g. irritable, hypotonic, tremors Features -short palpebral fissure -thin vermillion border/hypoplastic upper lip -smooth/absent filtrum -learning difficulties -microcephaly -growth retardation -epicanthic folds -cardiac malformations

Answer 23

The following should be recorded in all febrile children: -temperature -heart rate -respiratory rate -capillary refill time Signs of dehydration (reduced skin turgor, cool extremities etc) should also be looked for NB- print off feverish illness in children passmedicine

Answer 24

Features in males -learning difficulties -large low set ears, long thin face, high arched palate -macroorchidism -hypotonia -autism is more common -mitral valve prolapse ("fragile" mitral valve) Features in females (who have one fragile chromosome and one normal X chromosome) range from normal to mild Diagnosis -can be made antenatally by chorionic villus sampling or amniocentesis -genetic testing

Answer 25

Gastro-oesophageal reflux is the commonest cause of vomiting in infancy. Around 40% of infants regurgitate their feeds to a certain extent so there is a degree of overlap with normal physiological processes. Risk factors -preterm delivery -neurological disorders Features -typically develops before 8 weeks -vomiting/regurgitation following feeds Diagnosis is usually made clinically Complications -distress -failure to thrive -aspiration -frequent otitis media -in older children dental erosion may occur

Answer 26

Lifestyle- feed child with head up at 30 degrees, sleep on back, don't overfeed (smaller, more frequent feeds), thicker formulas if bottle-fed Medical- alginates (Gaviscon, not with thickeners), PPI if severe (see criteria below), rarely metoclopramide Specialist- refer to paediatrics if unsuccessful (may require surgery) NB- alginate before PPI. PPI criteria; -unexplained feeding difficulties (for example, refusing feeds, gagging or choking) -distressed behaviour -faltering growth NB- If there are severe complications (e.g. failure to thrive) and medical treatment is ineffective then fundoplication may be considered

Answer 27

A congenital defect in the anterior abdominal wall just lateral to the umbilical cord. The abdominal contents are NOT covered Management -vaginal delivery may be attempted -newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours

Answer 28

the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum. Associations -Beckwith-Wiedemann syndrome -Down's syndrome -cardiac and kidney malformations Management -caesarean section is indicated to reduce the risk of sac rupture -a staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure

Answer 29

Growing pains are equally common in boys and girls and occur in the age range of 3-12 years. Features of growing pains -never present at the start of the day after the child has woken -no limp -no limitation of physical activity -systemically well -normal physical examination -motor milestones normal -symptoms are often intermittent and worse after a day of vigorous activity -bilateral symptoms

Answer 30

pass medicine

Answer 31

Newborn babies are relatively deficient in vitamin K. This may result in impaired production of clotting factors which in turn can lead to haemorrhagic disease of the newborn (HDN). Bleeding may range from minor brushing to intracranial haemorrhages Breast-fed babies are particularly at risk as breast milk is a poor source of vitamin K. Maternal use of antiepileptics also increases the risk Because of this all newborns in the UK are offered vitamin K, either intramuscularly or orally

Answer 32

Criteria for immediate request for CT scan of the head (children); Loss of consciousness lasting more than 5 minutes (witnessed) Amnesia (antegrade or retrograde) lasting more than 5 minutes Abnormal drowsiness Three or more discrete episodes of vomiting Clinical suspicion of non-accidental injury Post-traumatic seizure but no history of epilepsy GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department Suspicion of open or depressed skull injury or tense fontanelle Any sign of basal skull fracture (haemotympanum, panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign) Focal neurological deficit If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object)

Answer 33

School exclusion is not advised for children with head lice fine-toothed combing of wet or dry hair treatment is only indicated if living lice are found a choice of treatments should be offered - malathion, wet combing, dimeticone, isopropyl myristate and cyclomethicone household contacts of patients with head lice do not need to be treated unless they are also affected

Answer 34

pass medicine

Answer 35

Migraine with aura

Answer 36

Caused by an aganglionic segment of bowel due to a developmental failure of the parasympathetic Auerbach and Meissner plexuses. Risk factors; -3 times more common in males -Down's syndrome Features; -neonatal period e.g. failure or delay to pass meconium -older children: constipation, abdominal distension Investigations; -abdominal x-ray -rectal biopsy: gold standard for diagnosis Management; -initially: rectal washouts/bowel irrigation -definitive management: surgery to affected segment of the colon NB- Hirschsprung-associated enterocolitis (HAEC) is 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

Answer 37

a congenital abnormality of the penis It is usually identified on the newborn baby check. If missed, parents may notice an abnormal urine stream. Hypospadias most commonly occurs as an isolated disorder. However, associated conditions include cryptorchidism (present in 10%) and inguinal hernia. Management; -refer to specialist -corrective surgery at 12 months (no circumcision prior, may be required in the repair) NB- in boys with very distal disease, no treatment may be needed.

Answer 38

The most common cause of hypothyroidism in children (juvenile hypothyroidism) is autoimmune thyroiditis. Other causes include -post total-body irradiation (e.g. in a child previous treated for acute lymphoblastic leukaemia) -iodine deficiency (the most common cause in the developing world)

Answer 39

Central causes -Down's syndrome -Prader-Willi syndrome -hypothyroidism -cerebral palsy (hypotonia may precede the development of spasticity) Neurological and muscular problems -spinal muscular atrophy -spina bifida -Guillain-Barre syndrome -myasthenia gravis -muscular dystrophy -myotonic dystrophy

Answer 40

an immune-mediated reduction in the platelet count. ITP in children is typically more acute than in adults and may follow an infection or vaccination ( ask about recent URTI) Features -bruising -petechial or purpuric rash -bleeding is less common and typically presents as epistaxis or gingival bleeding Investigation; - FBC (isolated thrombocytopenia) -blood film bone marrow examinations is only required if there are atypical features e.g. lymph node enlargement/splenomegaly, high/low white cells -failure to resolve/respond to treatment Management; usually, no treatment EXCLUDE MENINGITIS (RASH) advice to avoid activities that may result in trauma (and avoid NSAIDs, aspirin, blood thinners) other options may be indicated if the platelet count is very low (e.g. < 10 * 109/L) or there is significant bleeding. Options include: -oral/IV corticosteroid -IV immunoglobulins

Answer 41

Pass medicine

Answer 42

Infantile colic describes a relatively common and benign set of symptoms seen in young infants. It typically occurs in infants less than 3 months old and is characterised by bouts of excessive crying and pulling-up of the legs, often worse in the evening.

Answer 43

Ejection murmurs- due to turbulent blood flow at the outflow tract of the heart Venous hums- due to the turbulent blood flow in the great veins returning to the heart. Heard as a continuous blowing noise heard just below the clavicles Still's murmur- low-pitched sound heard at the lower left sternal edge Characteristics of an innocent ejection murmur include: Soft, Systolic, Short, Symptomless, Standing/Sitting (vary with position), Solitary site (no radiation)

Answer 44

The invagination of one portion of bowel into the lumen of the adjacent bowel, most commonly around the ileo-caecal region. Usually affects infants between 6-18 months old. Boys are affected twice as often as girls Features; -paroxysmal abdominal colic pain -during paroxysm the infant will characteristically draw their knees up and turn pale -vomiting -bloodstained stool - 'red-currant jelly' - is a late sign -sausage-shaped mass in the right upper quadrant Associated conditions: Concurrent viral illness Henoch-Schonlein purpura Cystic fibrosis Intestinal polyps Meckel diverticulum Investigation -USS is the investigation of choice (target-like mass) Management; -the majority of children can be treated with reduction by air insufflation under radiological control, which is now widely used first-line compared to the traditional barium enema -if this fails, or the child has signs of peritonitis, surgery is performed

Answer 45

First 24 hours- always pathological Causes of jaundice in the first 24 hrs -rhesus haemolytic disease -ABO haemolytic disease -hereditary spherocytosis -glucose-6-phosphodehydrogenase (G6PD) 2-14 days Jaundice in the neonate from 2-14 days is common (up to 40%) and usually physiological. It is due to a combination of factors, including more red blood cells, more fragile red blood cells and less developed liver function. More common if breastfed 14 days + (prolonged) If there are still signs of jaundice after 14 days (21 days if premature) a prolonged jaundice screen is performed, including: -conjugated and unconjugated bilirubin: the most important test as a raised conjugated bilirubin could indicate biliary atresia which requires urgent surgical intervention -direct antiglobulin test (Coombs' test) -TFTs -FBC and blood film -urine for MC&S and reducing sugars -U&Es and LFTs Causes of prolonged jaundice; -biliary atresia -hypothyroidism -galactosaemia -urinary tract infection -breast milk jaundice -prematurity -congenital infections e.g. CMV, toxoplasmosis

Answer 46

describes arthritis occurring in someone who is less than 16 years old that lasts for more than 6 weeks Pauciarticular JIA refers to cases where 4 or less joints are affected. It accounts for around 60% of cases of JIA Features of pauciarticular JIA -joint pain and swelling: usually medium sized joints e.g. knees, ankles, elbows -limp -ANA may be positive in JIA - associated with anterior uveitis Systemic onset JIA is a type of JIA which is also known as Still's disease Features of systemic onset JIA include -pyrexia -salmon-pink rash -lymphadenopathy -arthritis -uveitis -anorexia and weight loss Investigations -ANA may be positive, especially in oligoarticular JIA -rheumatoid factor is usually negative

Answer 47

a type of vasculitis which is predominately seen in children. Features -high-grade fever which lasts for > 5 days. Fever is characteristically resistant to antipyretics -conjunctival injection -bright red, cracked lips -strawberry tongue -cervical lymphadenopathy -red palms of the hands and the soles of the feet which later peel Clinical diagnosis Management -high-dose aspirin (one of the few indications for the use of aspirin in children. Due to the risk of Reye's syndrome aspirin is normally contraindicated in children) -intravenous immunoglobulin -echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms Complications -coronary artery aneurysm

Answer 48

Pass medicine

Answer 49

pass medicine

Answer 50

Features -precocious puberty -cafe-au-lait spots -polyostotic fibrous dysplasia -short stature

Answer 51

Features -prodromal phase -irritable -conjunctivitis -fever -Koplik spots (white spots ('grain of salt') on the buccal mucosa) -rash (starts behind ears then to the whole body discrete maculopapular rash becoming blotchy & confluent)- think M for Mastoid (&Measles- that's where it starts) -desquamation that typically spares the palms and soles may occur after a week -diarrhoea occurs in around 10% of patients Management -mainly supportive -admission may be considered in immunosuppressed or pregnant patients -notifiable disease → inform public health Complications -otitis media: the most common complication -pneumonia: the most common cause of death -encephalitis: typically occurs 1-2 weeks following the onset of the illness) -subacute sclerosing panencephalitis: very rare, may present 5-10 years following the illness -febrile convulsions -keratoconjunctivitis, corneal ulceration -diarrhoea -increased incidence of appendicitis -myocarditis

Answer 52

Osgood-Schlatter disease (tibial apophysitis) is a type of osteochondrosis characterised by inflammation at the tibial tuberosity. It is a traction apophysitis thought to be caused by repeated avulsion of the apophysis into which the patellar tendon is inserted Management is supportive

Answer 53

pass med print an algorithm

Answer 54

pass medicine

Answer 55

pass medicine

Answer 56

pass medicine

Answer 57

usually, the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance Features -left subclavicular thrill -continuous 'machinery' murmur -large volume, bounding, collapsing pulse -wide pulse pressure -heaving apex beat Management -indomethacin or ibuprofen to close PDA -if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair

Answer 58

usually, the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance Features -left subclavicular thrill -continuous 'machinery' murmur -large volume, bounding, collapsing pulse -wide pulse pressure -heaving apex beat Management -indomethacin or ibuprofen -if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair

Answer 59

A degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years. It is due to avascular necrosis of the femoral head, specifically the femoral epiphysis. Perthes' disease is 5 times more common in boys. Around 10% of cases are bilateral Features -hip pain: develops progressively over a few weeks limp (chronic, not acute) -stiffness and reduced range of hip movement -x-ray: early changes include widening of joint space, -later changes include decreased femoral head size/flattening NB- hyperactivity and short stature are associated Diagnosis -plain x-ray -technetium bone scan or magnetic resonance imaging if normal x-ray and symptoms persist Complications -osteoarthritis -premature fusion of the growth plates NB- Catterall staging Management -To keep the femoral head within the acetabulum: cast, braces -If less than 6 years: observation -Older: surgical management with moderate results -Operate on severe deformities Prognosis -Most cases will resolve with conservative management. Early diagnosis improves outcomes. Make sure to exclude SA (hip pain and fever in child- refer urgently)

Answer 60

Features -usually presents by 6 months e.g. with developmental delay -child classically has fair hair and blue eyes -learning difficulties -seizures, typically infantile spasms -eczema -'musty' odour to urine and sweat*

Answer 61

S .pneumoniae is the most likely causative agent of a bacterial pneumonia in children Management; -Amoxicillin is first-line for all children with pneumonia -Macrolides (erythro/clarithromycin) may be added if there is no response to first line therapy (should be used if mycoplasma or chlamydia is suspected) -In pneumonia associated with influenza, co-amoxiclav is recommended

Answer 62

Features -hypotonia during infancy -dysmorphic features -short stature -hypogonadism and infertility -learning difficulties -childhood obesity -behavioural problems in adolescence -Constant insatiable hunger that leads to obesity Management; Supportive- limit access to food, dieticians, occupational therapists, special educators Medical- GH (improves muscle development and body composition)

Answer 63

Usually viral induced (commonly RSV or rhinovirus) Other causes of wheeze; -asthma, bronchiolitis, FBA, CF, laryngomalacia, mediastinal mass Parents who are smokers should be strongly encouraged to stop. Episodic viral wheeze -treatment is symptomatic only -first-line is treatment with short acting beta 2 agonists (e.g. salbutamol) or anticholinergic via a spacer -next step is intermittent leukotriene receptor antagonist (montelukast), intermittent inhaled corticosteroids, or both NB- if severe, senior review, and very similar to asthma ladder treatment NB- Neither viral-induced wheeze or asthma cause a focal wheeze (they are usually widespread). If you hear a focal wheeze be very cautious and investigate further for a focal airway obstruction such as an inhaled foreign body or tumour. These patients will require an urgent senior review.

Answer 64

pass medicine

Answer 65

Moro Head extension causes abduction followed by adduction of the arms Present from birth to around 3-4 months of age Grasp Flexion of fingers when object placed in palm Present from birth to around 4-5 months of age Rooting Assists in breastfeeding Stroke cheek and they turn head in that direction and start sucking Present from birth to around 4 months of age Stepping Also known as walking reflex When child is held up and heels touch surface, legs will start marching Present from birth to around 2 months of age

Answer 66

Males -first sign is testicular growth at around 12 years of age (range = 10-15 years) -testicular volume > 4 ml indicates onset of puberty -maximum height spurt at 14 Females -first sign is breast development at around 11.5 years of age (range = 9-13 years) -height spurt reaches its maximum early in puberty (at 12) , before menarche -menarche at 13 (11-15) -there is an increase of only about 4% of height following menarche Normal changes in puberty -gynaecomastia may develop in boys -asymmetrical breast growth may occur in girls -diffuse enlargement of the thyroid gland may be seen

Answer 67

Pulmonary hypoplasia is a term used for newborn infants with underdeveloped lungs Causes include -oligohydramnios (eg. due to renal agenesis) -congenital diaphragmatic hernia

Answer 68

typically presents in the second to fourth weeks of life with vomiting, although rarely may present later at up to four months. It is caused by hypertrophy of the circular muscles of the pylorus. Features -'projectile' vomiting, typically 30 minutes after a feed -constipation and dehydration may also be present -a palpable mass may be present in the upper abdomen -hypochloraemic, hypokalaemic alkalosis due to persistent vomiting Diagnosis- USS Management- Ramstedt pyloromyotomy.

Answer 69

describes a syncopal episode (or presyncope) that occurs in response to pain or emotional stimuli. It is thought to be caused by neurally-mediated transient asystole in children with very sensitive vagal cardiac reflexes. It typically occurs in young children aged 6 months to 3 years Typical features; -child goes very pale -falls to floor -secondary anoxic seizures are common -rapid recovery There is no specific treatment and prognosis is excellent

Answer 70

The most common ocular malignancy found in children. The average age of diagnosis is 18 months. Autosomal dominant Features -absence of red-reflex, replaced by a white pupil (leukocoria) - the most common presenting symptom -strabismus -visual problems Management -enucleation is not the only option -other options include external beam radiation therapy, chemotherapy and photocoagulation Prognosis -excellent, with > 90% surviving into adulthood

Answer 71

a common disease of infancy caused by the human herpes virus 6 (HHV6). Features -high fever: lasting a few days, followed later by a -maculopapular rash -Nagayama spots: papular enanthem on the uvula and soft palate -febrile convulsions occur in around 10-15% -diarrhoea and cough are also commonly seen Other possible consequences of HHV6 infection -aseptic meningitis -hepatitis School exclusion is not needed.

Answer 72

it is an oral, live attenuated vaccine 2 doses are required, the first at 2 months, the second at 3 months the first dose should not be given after 14 weeks + 6 days and the second dose cannot be given after 23 weeks + 6 days due to a theoretical risk of intussusception

Answer 73

a reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes). Scarlet fever has an incubation period of 2-4 days and typically presents with: -fever: typically lasts 24 to 48 hours -malaise, headache, nausea/vomiting -sore throat -'strawberry' tongue -rash; fine punctate erythema ('pinhead') which generally appears first on the torso and spares the palms and soles children often have a flushed appearance with circumoral pallor. The rash is often more obvious in the flexures it is often described as having a rough 'sandpaper' texture desquamination occurs later in the course of the illness, particularly around the fingers and toes Diagnosis a throat swab is normally taken but antibiotic treatment should be commenced immediately, rather than waiting for the results Management -oral penicillin V for 10 days -patients who have a penicillin allergy should be given azithromycin -children can return to school 24 hours after commencing antibiotics -scarlet fever is a notifiable disease Scarlet fever is usually a mild illness but may be complicated by: -otitis media: the most common complication -rheumatic fever: typically occurs 20 days after infection -acute glomerulonephritis: typically occurs 10 days after infection -invasive complications (e.g. bacteraemia, meningitis, necrotizing fasciitis) are rare

Answer 74

Whooping cough- 2 days after commencing antibiotics (or 21 days from onset of symptoms if no antibiotics) Chickenpox- all lesions crusted over (usually takes 5 days) D&V- Until symptoms have settled for 48 hours Impetigo- Until lesions are crusted and healed, or 48 hours after commencing antibiotic treatment Influenza- Until recovered

Answer 75

Seborrhoeic dermatitis is a relatively common skin disorder seen in children. Due to a yeast that lives n our skin and is typically harmless. It typically affects the scalp ('Cradle cap'), nappy area, face and limb flexures. Cradle cap is an early sign which may develop in the first few weeks of life. It is characterised by an erythematous rash with coarse yellow scales. Management; -mild-moderate: baby shampoo and baby oils -severe: mild topical steroids e.g. 1% hydrocortisone Seborrheic dermatitis in children tends to resolve spontaneously by around 8 months of age

Answer 76

Symptoms; joint pain limp fever systemically unwell: lethargy Signs; swollen, red joint typically, only minimal movement of the affected joint is possible Investigations; joint aspiration: for culture. Will show a raised WBC raised inflammatory markers blood cultures The Kocher criteria for the diagnosis of septic arthritis: fever >38.5 degrees C non-weight bearing raised ESR raised WCC

Answer 77

Features which may be present in a sexually abused child; pregnancy sexually transmitted infections, recurrent UTIs sexually precocious behaviour anal fissure, bruising PV/PR bleeding reflex anal dilatation enuresis and encopresis behavioural problems, self-harm recurrent symptoms e.g. headaches, abdominal pain

Answer 78

This syndrome encompasses the triad of retinal haemorrhages, subdural haematoma, and encephalopathy. This is caused by the intentional shaking of a child (0-5 years old). The diagnosis of shaken baby syndrome has often made the headlines due to the controversy amongst physicians as to whether the mechanism of injury is definitely an intentional shaking of a child. This has often resulted in difficulty for the courts to convict suspects of causing shaken baby syndrome to a child.

Answer 79

A rare hip condition seen in children, classically seen in obese boys. It is also is known as slipped upper femoral epiphysis. NB- more common in obese children and boys Displacement of the femoral head epiphysis postero-inferiorly Features; hip, groin, medial thigh or knee pain loss of internal rotation of the leg in flexion bilateral slip in 20% of cases Investigation; AP and lateral (typically frog-leg) views are diagnostic Management; internal fixation

Answer 80

Croup Acute epiglottitis Inhaled foreign body Laryngomalacia

Answer 81

The commonest cause of death in the first year of life. It is most common at 3 months of age. Major risk factors; putting the baby to sleep prone parental smoking prematurity bed sharing hyperthermia (e.g. over-wrapping) or head covering (e.g. blanket accidentally moves) Other risk factors; male sex multiple births social classes IV and V maternal drug use incidence increases in winter NB- these odds are additive. Protective factors; breastfeeding room sharing (but not bed sharing, which is a significant risk factor) the use of dummies (pacifiers) Following a cot death siblings should be screened for potential sepsis and inborn errors of metabolism.

Answer 82

A condition seen in premature infants. It is caused by insufficient surfactant production and structural immaturity of the lungs Risk factors; prematurity male sex diabetic mothers Caesarean section second born of premature twins Features; tachypnoea intercostal recession expiratory grunting cyanosis NB- AKA respiratory distress Chest x-ray characteristically shows 'ground-glass' appearance with an indistinct heart border Management; prevention during pregnancy: maternal corticosteroids to induce fetal lung maturation oxygen assisted ventilation exogenous surfactant given via endotracheal tube

Answer 83

The most common cause of cyanotic congenital heart disease. It typically presents at around 1-2 months, although may not be picked up until the baby is 6 months old TOF is a result of anterior malalignment of the aorticopulmonary septum. The four characteristic features are: -ventricular septal defect (VSD) -right ventricular hypertrophy -right ventricular outflow tract obstruction, pulmonary stenosis -overriding aorta Features; cyanosis (expanded upon below) -unrepaired TOF infants may develop episodic hypercyanotic 'tet' spells due to near occlusion of the right ventricular outflow tract -features of tet spells include tachypnoea and severe cyanosis that may occasionally result in loss of consciousness -they typically occur when an infant is upset, is in pain or has a fever causes a right-to-left shunt ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually cause a murmur) a right-sided aortic arch is seen in 25% of patients chest x-ray shows a 'boot-shaped' heart, ECG shows right ventricular hypertrophy Management -surgical repair is often undertaken in two parts -cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm NB- the severity of the right ventricular outflow tract obstruction (pulmonary stenosis) determines the degree of cyanosis and clinical severity

Answer 84

Extremely common amongst children in the UK. Infestation occurs after swallowing eggs that are present in the environment. Threadworm infestation is asymptomatic in around 90% of cases, possible features include: -perianal itching, particularly at night -girls may have vulval symptoms Diagnosis may be made by the applying Sellotape to the perianal area and sending it to the laboratory for microscopy to see the eggs. However, most patients are treated empirically and this approach is supported in the CKS guidelines. Management -CKS recommend a combination of anthelmintic with hygiene measures for all members of the household -mebendazole is used first-line for children > 6 months old. A single dose is given unless infestation persists

Answer 85

It generally presents as acute hip pain following a recent viral infection. It is the commonest cause of hip pain in children. The typical age group is 3-8 years. Features; limp/refusal to weight bear groin or hip pain a low-grade fever (high fever- think septic arthritis) Management; Child with fever and limp/hip pain- same day assessment Transient synovitis is self-limiting, requiring only rest and analgesia.

Answer 86

The commonest cause of respiratory distress in the newborn period. It is caused by delayed resorption of fluid in the lungs It is more common following caesarean sections, possibly due to the lung fluid not being 'squeezed out' during the passage through the birth canal Chest x-ray may show hyperinflation of the lungs and fluid in the horizontal fissure. Management -observation, supportive care -supplementary oxygen may be required to maintain oxygen saturations Transient tachypnoea of the newborn usually settles within 1-2 days Difference between TTN and neonatal respiratory distress syndrome; Neonates with NRDS usually present with respiratory distress shortly after birth which usually worsens over the next few days. In contrast, TTN usually presents with tachypnoea shortly after birth and often fully resolves within the first day of life. A chest radiograph can be useful. In NRDS the characteristic features are a diffuse ground glass lungs with low volumes and a bell-shaped thorax. In TTN the CXR depicts a heart failure type pattern (e.g. interstitial oedema and pleural effusions) but key distinguishing features from congenital heart disease are a normal heart size and rapid resolution of the failure type pattern within days.

Answer 87

a form of cyanotic congenital heart disease. It is caused by the failure of the aorticopulmonary septum to spiral during septation. Children of diabetic mothers are at an increased risk of TGA. Clinical features; cyanosis tachypnoea loud single S2 prominent right ventricular impulse 'egg-on-side' appearance on chest x-ray Management; maintenance of the ductus arteriosus with prostaglandins surgical correction is the definite treatment.

Answer 88

A chromosomal disorder caused by either the presence of only one sex chromosome (X) or a deletion of the short arm of one of the X chromosomes. Turner's syndrome is denoted as 45,XO or 45,X. Features; short stature shield chest, widely spaced nipples webbed neck bicuspid aortic valve (15%), coarctation of the aorta (5-10%) primary amenorrhoea cystic hygroma (often diagnosed prenatally) high-arched palate short fourth metacarpal multiple pigmented naevi lymphoedema in neonates (especially feet) gonadotrophin levels will be elevated hypothyroidism is much more common in Turner's horseshoe kidney: the most common renal abnormality in Turner's syndrome NB- there is also an increased incidence of autoimmune disease (especially autoimmune thyroiditis) and Crohn's disease Management; Medical- GH (short stature), fertility treatments, oestrogen and progesterone (secondary sexual characteristics) NB- females with 1 X chromosome, this means they are more likely to exhibit X-linked recessive conditions eg. haemophillia

Answer 89

Complications of undescended testis -infertility -torsion -testicular cancer -psychological Management Unilateral undescended testis; -NICE CKS now recommend 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 Bilateral undescended testes -Should be reviewed by a senior paediatrician within 24hours as the child may need urgent endocrine or genetic investigation

Answer 90

Predisposing factors; Incomplete bladder emptying infrequent voiding hurried micturition obstruction by full rectum due to constipation neuropathic bladder Vesicoureteric reflux; a developmental anomaly found in around 35% of children who present with a UTI Poor hygiene; e.g. not wiping from front to back in girls Typical organisms- e coli, proteus, pseudomonas

Answer 91

the abnormal backflow of urine from the bladder into the ureter and kidney. It is a relatively common abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI), being found in around 30% of children who present with a UTI. As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI Possible presentations; -antenatal period: hydronephrosis on ultrasound -recurrent childhood urinary tract infections -reflux nephropathy (term used to describe chronic pyelonephritis secondary to VUR) Investigation; -micturating cystourethrogram -DMSA (nuclear medicine scan of kidney) scan: look for renal scarring

Answer 92

-infants: poor feeding, vomiting, irritability -younger children: abdominal pain, fever, dysuria -older children: dysuria, frequency, haematuria features which may suggest an upper UTI include: temperature > 38ºC, loin pain/tenderness

Answer 93

NICE guidelines for checking urine sample in a child; -if there are any symptoms or signs suggestive or a UTI -with unexplained fever of 38°C or higher (test urine after 24 hours at the latest) -with an alternative site of infection but who remain unwell (consider urine test after 24 hours at the latest) Urine collection method; -clean catch is preferable -if not possible then urine collection pads should be used -cotton wool balls, gauze and sanitary towels are not suitable -invasive methods such as suprapubic aspiration should only be used if non-invasive methods are not possible

Answer 94

-infants less than 3 months old should be referred immediately to a paediatrician -upper UTI- should be considered for admission to hospital. If not admitted oral antibiotics such as cephalosporin or co-amoxiclav should be given for 7-10 days -3+ months old with a lower UTI should be treated with oral antibiotics for 3 days according to local guidelines, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin (bring the children back if they remain unwell after 24-48 hours) NB- antibiotic prophylaxis should be considered with recurrent UTIs

Answer 95

an infectious disease caused by the Gram-negative bacterium Bordetella pertussis. It typically presents in children Immunisation; -2, 3, 4 months and 3-5 years. Newborn infants are particularly vulnerable- pregnant women vaccinated -neither infection nor immunisation results in lifelong protection (adolescents and adults may develop whooping cough) Features; -2-3 days of coryza precede onset of: -coughing bouts: usually worse at night and after feeding, may be ended by vomiting & associated central cyanosis -inspiratory whoop: not always present (caused by forced inspiration against a closed glottis) -infants may have spells of apnoea -persistent coughing may cause subconjunctival haemorrhages or even anoxia leading to syncope & seizures -symptoms may last 10-14 weeks* and tend to be more severe in infants -marked lymphocytosis Diagnosis; -per nasal swab culture for Bordetella pertussis (days-weeks) -PCR and serology Management; Infants under 6 months with suspect pertussis should be admitted in the UK pertussis is a notifiable disease an oral macrolide (e.g. clarithromycin, azithromycin or erythromycin) is indicated if the onset of the cough is within the previous 21 days to eradicate the organism and reduce the spread household contacts should be offered antibiotic prophylaxis antibiotic therapy has not been shown to alter the course of the illness school exclusion: 48 hours after commencing antibiotics (or 21 days from onset of symptoms if no antibiotics )

Answer 96

an inherited neurodevelopmental disorder caused by a microdeletion on chromosome 7 Features; elfin-like facies characteristic like affect - very friendly and social learning difficulties short stature transient neonatal hypercalcaemia supravalvular aortic stenosis Diagnosis is made by FISH studies NB- the distinctive features to remember with William syndrome are the very sociable personality, the starburst eyes and the wide mouth with a big smile. It is worth remembering the association with supravalvular aortic stenosis and hypercalcaemia Management; Supportive- physiotherapists, social workers, occupational therapists, educational support, monitoring for cardiac complications Medical- low calcium diet

Answer 97

one of the most common childhood malignancies. It typically presents in children under 5 years of age Associations; Beckwith-Wiedemann syndrome as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation hemihypertrophy Features; abdominal mass (most common presenting feature) painless haematuria scrotal swelling flank pain other features: anorexia, fever unilateral in 95% of cases metastases are found in 20% of patients (most commonly lung) Referral; children with an unexplained enlarged abdominal mass in children - possible Wilm's tumour - arrange paediatric review with 48 hours Management; nephrectomy chemotherapy radiotherapy if advanced disease prognosis: good, 80% cure rate

Answer 98

Becker muscular dystrophy Colour blindness Duchenne muscular dystrophy G6PD deficiency Haemophilia A,B Nephrogenic diabetes insipidus

Answer 99

Rising pCO2- airways have collapsed and can’t clear waste carbon dioxide Falling pH- CO2 is building up and they are not able to buffer the acidosis this creates. This is a respiratory acidosis. If they are also hypoxic, this is classed as type 2 respiratory failure.

Answer 100

a congenital abnormality that predisposes to dynamic supraglottic collapse during the inspiratory phase of respiration, resulting in intermittent upper airway obstruction and stridor. Features; Usually happy and thriving infants Inspiratory stridor: worsens in supine position, during crying, upper respiratory tract infections, agitation, and feeding (the noise steadily increases over the first 6 months, as inspiratory airflow increases with age) Reflux may be present (as a result of higher intrathoracic pressures necessary to overcome the inspiratory obstruction) Failure to thrive and sleep-disordered breathing in severe cases NB- symptoms begin within the first 2 months of life and peak at 6–8 months NB- no respiratory distress Investigations- bronchoscopy Management; 90% resolve Some need surgery

Answer 101

Used to describe Hypotension, Bronchoconstriction or Airway compromise in the setting of an allergic reaction Clinical diagnosis (but serum tryptase at 6 hours can hep confirm whether anaphylaxis occurred or not) Management; 1- A-E assessment 2- 3 medications must be given; intramuscular adrenalin, repeated after 5 minutes if required, antihistamines, such as oral chlorphenamine or cetirizine, and steroids NB- need to know doses of the 3 drugs (wont have time to look up in an emergency)

Answer 102

Ammonia glucose and gas

Answer 103

Always treat as sepsis until proven otherwise

Answer 104

S. aureus is the most common cause of recurrent pulmonary infection in infancy and childhood. P. aeruginosa is the most common cause of recurrent pulmonary infections in adulthood.

Answer 105

encompasses the triad of retinal haemorrhages, subdural haematoma, and encephalopathy

Answer 106

Excessive weight loss Amenorrhoea Lanugo hair- fine, soft hair across most of the body (and then scalp hair loss/ thinning) Hypokalaemia Hypotension Hypothermia, cold peripheries Constipation Dry skin Lethargy, tiredness Peripheral oedema Psychosocial Cardiac complications include arrhythmia (bradycardia), cardiac atrophy, and sudden cardiac death Physiological Abnormalities; Hypokalaemia Low FSH, LH, oestrogens, and testosterone Raised cortisol and growth hormone Hypercholesterolaemia NB- always rule out coeliac and malignancy NB- most things low, G's and C's raised: growth hormone, glucose, salivary glands, cortisol, cholesterol, carotinaemia

Answer 107

1. Short term- physical stabilisation, thiamine, Vitamin B complex and multivitamins, diet plan 2. MDT approach- child psychiatrist diagnosis, treatment of co-morbidities eg. anxiety, dietician input, support from community mental health team, paediatrician to monitor physical health eg. DEXA scan for bone mineral density 3. Long term- prevention of relapse, continuing support etc. eg. BEAT charity

Answer 108

Normal body weight (usually) Alkalosis, due to vomiting hydrochloric acid from the stomach Hypokalaemia Erosion of teeth Swollen salivary glands Mouth ulcers Gastro-oesophageal reflux and irritation Calluses on the knuckles where they have been scraped across the teeth. This is called Russell’s sign.

Answer 109

Coeliac disease Type 1 diabetes mellitus Hyperthyroidism Malignancy Anorexia nervosa Inflammatory bowel disease Oesophageal problems eg achalasia Severe depression/OCD/autism Juvenile arthritis Addison's

Answer 110

Features; Frequent regurgitation progressing to projectile, nonbilious vomiting immediately after feeding An enlarged, thickened, "olive-shaped”, non-tender pylorus (diameter of 1–2 cm) should be palpable in the epigastrium A peristaltic wave, moving from left to right, may be evident in the epigastrium "Hungry vomiter": demands re-feeding after vomiting; demonstrates a strong rooting and sucking reflex, irritable If left untreated: dehydration, weight loss, failure to thrive Blood gas analysis will show a hypochloric (low chloride) metabolic alkalosis as the baby is vomiting the hydrochloric acid from the stomach. NB- USS is investigation of choice (thickened pylorus) Management; Supportive- IV fluids Surgical- laparoscopic pyloromyotomy

Answer 111

a potentially serious acute skin condition caused by the exfoliative toxins of Staphylococcus aureus and typically affects infants and young children (history will usually point to a local staph infection eg. pharyngitis or bullous impetigo) NB- Stevens-Johnson syndrome and toxic epidermal necrolysis manifest with mucosal involvement, SSSS does not

Answer 112

a rare toxin-mediated life-threatening acute condition caused by toxin-producing strains of Streptococcus pyogenes and Staphylococcus aureus (MRSA) Features; fever: temperature > 38.9ºC hypotension: systolic blood pressure < 90 mmHg diffuse erythematous rash desquamation of rash, especially of the palms and soles involvement of three or more organ systems: e.g. gastrointestinal (diarrhoea and vomiting), mucous membrane erythema, renal failure, hepatitis, thrombocytopenia, CNS involvement (e.g. confusion)

Answer 113

VTE Cerebral oedema Hypokalaemia

Answer 114

Thyroid disease at diagnosis and annually thereafter until transfer to adult services Diabetic retinopathy- annually from 12 years Moderately increased albuminuria (albumin:creatinine ratio [ACR] 3–30 mg/mmol; ‘microalbuminuria’) to detect diabetic kidney disease- annually from 12 years Hypertension- annually from 12 years.

Answer 115

involuntary episodes during which a child holds their breath, usually triggered by something upsetting or scaring them. They typically occur between 6 and 18 months of age. The child has no control over the breath holding spells. They are not harmful in the long term, do not lead to epilepsy and most children outgrow them by 4 or 5 years. They are often divided into two types: cyanotic breath holding spells and pallid breath holding spells (also known as reflex anoxic seizures). Management; After excluding other pathology and making a diagnosis, educating and reassuring parents about breath holding spells is the key to management. Breath holding spells have been linked with iron deficiency anaemia. Treating the child if they are iron deficiency anaemic can help minimise further episodes.

Answer 116

Cyanotic breath holding spells occur when the child is really upset, worked up and crying. After letting out a long cry they stop breathing, become cyanotic and lose consciousness. Within a minute they regain consciousness and start breathing. They can be a bit tired and lethargic after an episode. Reflex anoxic seizures occur when the child is startled. The vagus nerve sends strong signals to the heart that causes it to stop beating. The child will suddenly go pale, lose consciousness and may start to have some seizure-like muscle twitching. Within 30 seconds the heart restarts and the child becomes conscious again.

Answer 117

describes cerebrospinal fluid (CSF) building up abnormally within the brain and spinal cord. This is a result of either over-production of CSF or a problem with draining or absorbing CSF. Congenital Causes; The most common cause of hydrocephalus is aqueductal stenosis, leading to insufficiency drainage of CSF. The cerebral aqueduct that connects the third and fourth ventricle is stenosed (narrowed). This blocks the normal flow of CSF out of the third ventricle, causing CSF to build up in the lateral and third ventricles. Other causes: Arachnoid cysts can block the outflow of CSF if they are large enough Arnold-Chiari malformation is where the cerebellum herniates downwards through the foramen magnum, blocking the outflow of CSF Chromosomal abnormalities and congenital malformations can cause obstruction to CSF drainage. Presentation; -enlarged and rapidly increasing head circumference (occipito-frontal circumference). -Bulging anterior fontanelle -Poor feeding and vomiting -Poor tone -Sleepiness Definitive management- VP shunt Complications of a VP Shunt; -Infection -Blockage -Excessive drainage -Intraventricular haemorrhage during shunt related surgery -Outgrowing them (they typically need replacing around every 2 years as the child grows)

Answer 118

a rare autosomal recessive condition that causes a progressive loss of motor neurones, leading to progressive muscular weakness. Spinal muscular atrophy affects the lower motor neurones in the spinal cord. This means there will be lower motor neurone signs, such as fasciculations, reduced muscle bulk, reduced tone, reduced power and reduced or absent reflexes. Management (no cure) -MDT approach -Supportive- physiotherapy, mobility aids, NIV -Medical/surgical- tracheostomy or PEG insertion

Answer 119

SCID will present in the first few months of life with: Persistent severe diarrhoea Failure to thrive Opportunistic infections that are more frequent or severe than in healthy children, for example severe and later fatal chickenpox, Pneumocystis jiroveci pneumonia and cytomegalovirus Unwell after live vaccinations such as the BCG, MMR and nasal flu vaccine Omenn syndrome (see below) classic features of Omenn syndrome: A red, scaly, dry rash (erythroderma) Hair loss (alopecia) Diarrhoea Failure to thrive Lymphadenopathy Hepatosplenomegaly Management (fatal unless successfully treated) -supportive- treat any infections, sterile environment, avoid live vaccines -medical- immunoglobulin therapy -surgical- haematopoietic stem cell transplantation

Answer 120

also called 22q11.2 deletion syndrome, results from a microdeletion in a portion of chromosome 22 that leads to a developmental defect in the third pharyngeal pouch and third branchial cleft. One of the consequences of this is incomplete development of the thymus gland. An underdeveloped thymus gland results in an inability to create functional T cells. Features of DiGeorge syndrome can be remembered with the CATCH-22 mnemonic: C – Congenital heart disease A – Abnormal facies (characteristic facial appearance) T – Thymus gland incompletely developed C – Cleft palate H – Hypoparathyroidism and resulting Hypocalcaemia 22nd chromosome affected

Answer 121

Presentation; Episodic symptoms with intermittent exacerbations Diurnal variability, typically worse at night and early morning Dry cough with wheeze and shortness of breath Typical triggers A history of other atopic conditions such as eczema, hayfever and food allergies Family history of asthma or atopy Bilateral widespread “polyphonic” wheeze heard by a healthcare professional Symptoms improve with bronchodilators Presentation Indicating a Diagnosis Other Than Asthma; Wheeze only related to coughs and colds, more suggestive of viral induced wheeze Isolated or productive cough Normal investigations No response to treatment Unilateral wheeze suggesting a focal lesion, inhaled foreign body or infection Typical Triggers; Dust (house dust mites) Animals Cold air Exercise Smoke Food allergens (e.g. peanuts, shellfish or eggs) Investigations; NB- typically clinical diagnosis, but the below can help; -Spirometry with reversibility testing (in children aged over 5 years) -Direct bronchial challenge test with histamine or methacholine -Fractional exhaled nitric oxide (FeNO) -Peak flow variability measured by keeping a diary of peak flow measurements several times a day for 2 to 4 weeks

Answer 122

Medical Therapy in Under 5 Years; 1. SABA 2. ICS + SABA 3. ICS + SABA + LTRA 4. Stop LTRA and refer to specialist Medical Therapy 5-16; 1. SABA 2. ICS + SABA 3. ICS + SABA + LTRA 4. ICS + SABA + LABA NB- yearly review, vaccinations, always check inhaler technique and adherence at each review etc. (same stuff as for adults)

Answer 123

Presentation; Progressively worsening shortness of breath Signs of respiratory distress Fast respiratory rate (tachypnoea) Expiratory wheeze on auscultation heard throughout the chest The chest can sound “tight” on auscultation, with reduced air entry NB- A silent chest is an ominous sign. This is where the airways are so tight it is not possible for the child to move enough air through the airways to create a wheeze. Severity- see print out Staples of management in acute viral induced wheeze or asthma are: -Supplementary oxygen if required (i.e. oxygen saturations less than 94% or working hard) -Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate) -Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous) -Antibiotics only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin) NB- If you haven’t got control after working down the ladder the situation is very serious. Call an anaesthetist and the intensive care unit. They may need intubation and ventilation. This call should be made earlier to give the best chance of successfully intubating them before the airway becomes too constricted. NB- Consider monitoring the serum potassium when on high doses of salbutamol as it causes potassium to be absorbed from the blood into the cells. Look for evidence of cyanosis (central or peripheral), tracheal tug, subcostal recessions, hypoxia, tachypnoea or wheeze on auscultation.

Answer 124

Generally, discharge can be considered when the child well on 6 puffs 4 hourly of salbutamol. They can be prescribed a reducing regime of salbutamol to continue at home, for example 6 puffs 4 hourly for 48 hours then 4 puffs 6 hourly for 48 hours then 2-4 puffs as required. A few other steps to consider: Finish the course of steroids if these were started (typically 3 days total) Provide safety-net information about when to return to hospital or seek help Provide an individualised written asthma action plan

Answer 125

Medical Causes of Abdominal Pain Functional Constipation is also very common Urinary tract infection Coeliac disease Inflammatory bowel disease Irritable bowel syndrome Mesenteric adenitis Abdominal migraine Pyelonephritis Henoch-Schonlein purpura Tonsilitis Diabetic ketoacidosis Infantile colic There are addition causes in adolescent girls: Dysmenorrhea (period pain) Mittelschmerz (ovulation pain) Ectopic pregnancy Pelvic inflammatory disease Ovarian torsion Pregnancy Surgical Causes of Abdominal Pain Appendicitis causes central abdominal pain spreading to the right iliac fossa Intussusception causes colicky non-specific abdominal pain with redcurrant jelly stools Bowel obstruction causes pain, distention, absolute constipation and vomiting Testicular torsion causes sudden onset, unilateral testicular pain, nausea and vomiting Red Flags for Serious Abdominal Pain Persistent or bilious vomiting Severe chronic diarrhoea Fever Rectal bleeding Weight loss or faltering growth Dysphagia (difficulty swallowing) Nighttime pain Abdominal tenderness Initial investigations that may indicate the pathology pathology: Anaemia can indicate inflammatory bowel disease or coeliac disease Raised inflammatory markers (ESR and CRP) can indicate inflammatory bowel disease Raised anti-TTG or anti-EMA antibodies indicates coeliac disease Raised faecal calprotectin indicates inflammatory bowel disease Positive urine dipstick indicates a urinary tract infection

Answer 126

A diagnosis of recurrent abdominal pain is made when a child presents with repeated episodes of abdominal pain without an identifiable underlying cause. The pain is described as non-organic or functional. This is common and can lead to psychosocial problems, such as missed days at school and parental anxiety. There is overlap between the diagnoses of recurrent abdominal pain, abdominal migraine, irritable bowel syndrome and functional abdominal pain. Recurrent abdominal pain often corresponds to stressful life events, such as loss of a relative or bullying. The leading theory for the cause is increased sensitivity and inappropriate pain signals from the visceral nerves (the nerves in the gut) in response to normal stimuli. Management involves careful explanation and reassurance. Measures that can help manage the pain are: Distracting the child from the pain with other activities or interests Encourage parents not to ask about or focus on the pain Advice about sleep, regular meals, healthy balanced diet, staying hydrated, exercise and reducing stress Probiotic supplements may help symptoms of irritable bowel syndrome Avoid NSAIDs such as ibuprofen Address psychosocial triggers and exacerbating factors Support from a school counsellor or child psychologist

Answer 127

Down’s syndrome Fragile X syndrome Fetal alcohol syndrome Rett syndrome Metabolic disorders

Answer 128

Dyspraxia Cerebral palsy Muscular dystrophy Visual impairment Congenital ataxia (rare)

Answer 129

Cerebral palsy Ataxia Myopathy Spina bifida Visual impairment

Answer 130

Specific social circumstances, for example exposure to multiple languages or siblings that do all the talking Hearing impairment Learning disability Neglect Autism Cerebral palsy

Answer 131

Emotional and social neglect Parenting issues Autism

Answer 132

Keep looking at 0-final notes printed, not all of them have been made into flashcards

Answer 133

classic triad of: -Haemolytic anaemia -Acute kidney injury -Thrombocytopenia The most common cause is a toxin produced by the e. coli 0157 bacteria, called the shiga toxin. Shigella also produces this toxin. The use of antibiotics and anti-motility medications such as loperamide to treat gastroenteritis caused by these pathogens increases the risk of developing HUS. Presentation; E. coli 0157 causes a brief gastroenteritis, often with bloody diarrhoea. The symptoms of haemolytic uraemic syndrome typically start around 5 days after the onset of the diarrhoea. Signs and symptoms of HUS may include: Reduced urine output Haematuria or dark brown urine Abdominal pain Lethargy and irritability Confusion Oedema Hypertension Bruising Management; HUS is a medical emergency and has a 10% mortality. It needs to be managed by experienced paediatricians under the guidance of a renal specialist. The condition is self limiting and supportive management is the mainstay of treatment: -Urgent referral to the paediatric renal unit for renal dialysis if required -Antihypertensives if required -Careful maintenance of fluid balance -Blood transfusions if required

Answer 134

Anything that leads to inadequate energy and nutrition can lead to failure to thrive. The causes can be categorised as: -Inadequate nutritional intake -Difficulty feeding -Malabsorption -Increased energy requirements -Inability to process nutrition Causes of Inadequate Nutritional Intake Maternal malabsorption if breastfeeding Iron deficiency anaemia Family or parental problems Neglect Availability of food (i.e. poverty) Causes of Difficulty Feeding Poor suck, for example due to cerebral palsy Cleft lip or palate Genetic conditions with an abnormal facial structure Pyloric stenosis Causes of Malabsorption Cystic fibrosis Coeliac disease Cows milk intolerance Chronic diarrhoea Inflammatory bowel disease Causes of Increased Energy Requirements Hyperthyroidism Chronic disease, for example congenital heart disease and cystic fibrosis Malignancy Chronic infections, for example HIV or immunodeficiency Inability to Process Nutrients Properly Inborn errors of metabolism Type 1 diabetes Investigations; -Urine dipstick, for urinary tract infection -Coeliac screen (anti-TTG or anti-EMA antibodies) -Further investigations are usually not necessary where there are no other clinical concerns. Focused investigations should be considered where additional signs or symptoms suggest an underlying diagnosis, such as cystic fibrosis or pyloric stenosis.

Answer 135

Management depends on the cause and may involve input from the multidisciplinary team. All children with faltering growth should have regular reviews to monitor weight gain. Reviews that are too frequent can increase parental anxiety. Where difficulty with breastfeeding is the cause, there are lots of ways for the mother to get support, including midwives, health visitors, peers groups and “lactation consultants”. Supplementing with formula milk is likely to successfully improve growth, however it often results in breastfeeding stopping. Mother should be encouraged to feed with breastmilk prior to top-up feeds, and express when not breastfeeding to encourage lactation to continue. Where inadequate nutrition is the cause there are several management options based on individual circumstances: Encouraging regular structured mealtimes and snacks Reduce milk consumption to improve appetite for other foods Review by a dietician Additional energy dense foods to boost calories Nutritional supplements drinks Where other measures fail and there are serious concerns the multidisciplinary team may consider enteral tube feeding. This needs to have clear goals and a defined end point.

Answer 136

Recurrent UTIs should be investigated for an underlying cause and renal damage. This is a summary of the NICE guidelines on urinary tract infections in under 16s. Please read the full guidelines before treating patients. Ultrasound Scans -All children under 6 months with their first UTI should have an abdominal ultrasound within 6 weeks, or during the illness if there are recurrent UTIs or atypical bacteria -Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks -Children with atypical UTIs should have an abdominal ultrasound during the illness DMSA (Dimercaptosuccinic Acid) Scan DMSA scans should be used 4 – 6 months after the illness to assess for damage from recurrent or atypical UTIs. This involves injecting a radioactive material (DMSA) and using a gamma camera to assess how well the material is taken up by the kidneys. Where there are patches of kidney that have not taken up the material, this indicates scarring that may be the result of previous infection. Vesico-Ureteric Reflux (VUR) Vesico-ureteric reflux (VUR) is where urine has a tendency to flow from the bladder back into the ureters. This predisposes patients to developing upper urinary tract infections and subsequent renal scarring. This is diagnosed using a micturating cystourethrogram (MCUG). Management of vesico-ureteric reflux depends on the severity: Avoid constipation Avoid an excessively full bladder Prophylactic antibiotics Surgical input from paediatric urology Micturating Cystourethrogram (MCUG) Micturating cystourethrogram (MCUG) should be used to investigate atypical or recurrent UTIs in children under 6 months. It is also used where there is a family history of vesico-ureteric reflux, dilatation of the ureter on ultrasound or poor urinary flow. A MCUG is used to diagnose VUR. It involves catheterising the child, injecting contrast into the bladder and taking a series of xray films to determine whether the contrast is refluxing into the ureters. Children are usually given prophylactic antibiotics for 3 days around the time of the investigation.

Answer 137

Raised respiratory rate Use of accessory muscles of breathing, such as the sternocleidomastoid, abdominal and intercostal muscles Intercostal and subcostal recessions Nasal flaring Head bobbing Tracheal tugging Cyanosis (due to low oxygen saturation) Abnormal airway noises Abnormal Airway Noises -Wheezing is a whistling sound caused by narrowed airways, typically heard during expiration -Grunting is caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure (NB- isolated grunting in an unwell child is more likely to indicate sepsis rather than bronchiolitis) -Stridor is a high pitched inspiratory noise caused by obstruction of the upper airway, for example in croup

Answer 138

An autosomal recessive condition affecting the cilia of various cells in the body. PCD causes dysfunction of the motile cilia around the body, most notably in the respiratory tract NB- there is a strong link between primary ciliary dyskinesia and situs inversus. Kartagner’s Triad; Kartagner’s triad describes the three key features of PCD. Not all patients will have all three features. These are: -Paranasal sinusitis -Bronchiectasis -Situs Inversus Investigations; -CXR (situs invertus and bronchiectasis) -Bronchoscopy with biopsy -Semen analysis (male infertility) Management; Similar to cystic fibrosis and bronchiectasis with daily physiotherapy, a high calorie diet and antibiotics.

Answer 139

Suspect inflammatory bowel disease in children and teenagers presenting with perfuse diarrhoea, abdominal pain, bleeding, weight loss or anaemia. They may be systemically unwell during flares, with fevers, malaise and dehydration. See 3rd year notes for more detail (management/ investigations etc.)

Answer 140

Where a physical obstruction prevents the flow of faeces through the intestines. This blockage will lead to a back-pressure through the gastrointestinal system, causing vomiting. It also causes absolute constipation, where the patient is unable to pass stools or wind. Causes; Meconium ileus Hirschsprung’s disease Oesophageal atresia Duodenal atresia Intussusception Imperforate anus (ARM) Malrotation of the intestines with a volvulus Strangulated hernia Features; -Persistent vomiting. This may be bilious, containing bright green bile. -Abdominal pain and distention -Failure to pass stools or wind -Abnormal bowel sounds. These can be high pitched and “tinkling” early in the obstruction and absent later. Investigations; Bedside- obs, exam Blood- metabolic panel etc. Specialist- Abdominal X-ray (dilated loops, absence of air in rectum) Management; Conservative- nil by mouth (NG tube), IV fluids Medical- analgesia Surgical- repair underlying cause (if necessary)

Answer 141

Congenital growth hormone deficiency- congenital underproduction Acquired growth hormone deficiency- secondary to infection, trauma or interventions such as surgery NB- can occur in isolation or in combination with other pituitary hormone deficiencies like hypothyroidism, adrenal insufficiency Features; Growth hormone deficiency may present at birth or in neonates with: -Micropenis (in males) -Hypoglycaemia -Severe jaundice Older infants and children can present with: -Poor growth, usually stopping or severely slowing from age 2-3 -Short stature -Slow development of movement and strength -Delayed puberty Investigations; Bedside- Bloods- other pituitary deficiencies (thyroid/adrenal) Specialist- GH stimulation test (poor response), genetic counselling (if necessary), MRI head (pituitary/hypothalamus abnormalities), X-ray or DEXA (bone age, determine final height) Management; Conservative- paediatric endocrinology referral Medical- s/c injections of GH (somatropin)

Answer 142

Where there is tissue at the proximal end of the urethra (closest to the bladder) that causes obstruction of urine output. The obstruction to the outflow of urine causes hydronephrosis. A restriction in the outflow of urine prevents the bladder from fully emptying, leading to a reservoir of urine that increases the risk of urinary tract infections. Features; Difficulty urinating Weak urinary stream Chronic urinary retention Palpable bladder Recurrent urinary tract infections Impaired kidney function NB- severe cases can cause bilateral hydronephrosis in the foetus and oligohydramnios. The oligohydramnios leads to underdeveloped fetal lungs (pulmonary hypoplasia) with respiratory failure shortly after birth. Investigations; NB- usually picked up on antenatal scans (bilateral hydronephrosis) Bedside- Bloods- Specialist- abdominal USS, micturating cystourethrogram (MCUG),Cystoscopy Management; Conservative- watchful waiting, urinary catheter Surgical- ablation of tissue (during cystoscopy)

Answer 143

Simple hydroceles are common in newborn males. They occurs where fluid is trapped in the tunica vaginalis. Usually this fluid gets reabsorbed over time and the hydrocele disappears. The key differential diagnoses of a scrotal or inguinal swelling in a neonate are: Hydrocele Partially descended testes Inguinal hernia Testicular torsion Haematoma Tumours (rare) Management; -USS -Simple hydroceles will resolve, communicating: surgery

Answer 144

Simple hydroceles are common in newborn males. They occurs where fluid is trapped in the tunica vaginalis. Usually this fluid gets reabsorbed over time and the hydrocele disappears. The key differential diagnoses of a scrotal or inguinal swelling in a neonate are: Hydrocele Partially descended testes Inguinal hernia Testicular torsion Haematoma Tumours (rare) Management; -USS -Simple hydroceles will resolve, communicating: surgery

Answer 145

see previous notes

Answer 146

see previous notes

Answer 147

Features Short stature Broad forehead Downward sloping eyes with ptosis Hypertelorism (wide space between the eyes) Prominent nasolabial folds Low set ears Webbed neck Widely spaced nipples Associated Conditions Congenital heart disease, particularly pulmonary valve stenosis, hypertrophic cardiomyopathy and ASD Cryptorchidism (undescended testes) can lead to infertility. Fertility is normal in women. Learning disability Bleeding disorders Lymphoedema Increased risk of leukaemia and neuroblastoma Management; Conservative- supportive, MDT, monitor cardiac complications

Answer 148

Fragile X syndrome usually presents with a delay in speech and language development. Other features are: Intellectual disability Long, narrow face Large ears Large testicles after puberty Hypermobile joints (particularly in the hands) Attention deficit hyperactivity disorder (ADHD) Autism Seizures Management; Conservative- supportive, deal with autism and ADHD (special school etc.)

Answer 149

Features; Delayed development and learning disability Severe delay or absence of speech development Coordination and balance problems (ataxia) Fascination with water Happy demeanour Inappropriate laughter Hand flapping Abnormal sleep patterns Epilepsy Attention-deficit hyperactivity disorder Dysmorphic features Microcephaly Fair skin, light hair and blue eyes Wide mouth with widely spaced teeth Management; Supportive- physiotherapists, social workers, occupational therapists, educational support

Answer 150

See zero to finals (need to go over- MH can come up in F&C (also with postnatal depression))

Answer 151

Causes in infancy; -Physiological anaemia -Anaemia of prematurity -Blood loss -Haemolysis (haemolytic disease of newborn, Hereditary spherocytosis, G6PD deficiency) -Twin-twin transfusion, where blood is unequally distributed between twins that share a placenta Causes in older children; -Iron deficiency anaemia secondary to dietary insufficiency. This is the most common cause overall -Malabsorption ie. coeliac, IBD (can be Fe,B12, or folate anaemia) -Blood loss, most frequently from menstruation in older girls -Sickle cell anaemia -Thalassaemia -Leukaemia -Hereditary spherocytosis -Hereditary eliptocytosis -Sideroblastic anaemia Symptoms of anaemia Tiredness Shortness of breath Headaches Dizziness Palpitations Worsening of other conditions Pica, hair loss (iron-deficiency specific) Signs of anaemia; Pale skin Conjunctival pallor Tachycardia Raised respiratory rate Signs of specific causes of anaemia: Koilonychia refers to spoon shaped nails, which can indicate iron deficiency Angular chelitis can indicate iron deficiency Atrophic glossitis is a smooth tongue due to atrophy of the papillae and can indicate iron deficiency Brittle hair and nails can indicate iron deficiency Jaundice occurs in haemolytic anaemia Bone deformities occur in thalassaemia Investigations; Bedside- observations, physical examination Bloods- FBC, blood film, haemanitics, reticulocyte count LFT (bilirubin), direct coombs (autoimmune haemolytic), haemoglobin electrophoresis (haemoglobinopathies) Specialist- NB- increased reticulocytes: due to haemolysis or blood loss.

Answer 152

a viral infection spread by respiratory droplets Features; Fever Muscle aches Lethargy Reduced appetite Headache Dry mouth Parotid gland swelling, either unilateral or bilateral, with associated pain is the key feature that should make you consider mumps. It can also present with symptoms of the complications, such as: Abdominal pain (pancreatitis) Testicular pain and swelling (orchitis) Confusion, neck stiffness and headache (meningitis or encephalitis) Investigations and Management; -PCR testing on a saliva swab -Supportive management (rest, fluid, analgesia, self-limiting) Complications; Complications Pancreatitis Orchitis (most common in post-pubertal males) Meningitis Sensorineural hearing loss

Answer 153

See ENT notes

Answer 154

see previous notes

Answer 155

see previous notes NB- go through 0-finals before exams (some notes will be in sections from M&M that I may have missed)

Answer 156

ITP HSP HUS (low platelets causes the rash, after diarrhoeal illness) NAI Meningococcal septicaemia Haematological malignancy (low platelets) DIC (due to sepsis) Forceful coughing or vomiting

Answer 157

Needed if patient cannot meet fluid needs orally (eg. NBM) Holliday-Segar formula; (this is total volume needed in 24 hours); 100ml/kg for first 10kg 50ml/kg for next 10kg 20ml/kg for anything over 20kg Rate of infusion = total amount in ml / 24 NB- fluid of choice: 0.9% sodium chloride + 5% glucose

Answer 158

%dehydration x weight (kg) x 10 = mls of fluid to replace %dehydration = well weight-current weight/ well weight x 100

Answer 159

Total fluid in 24 hours (ml) = routine maintenance + replacement fluid Rate of infusion (ml/hr) = total amount (ml) / 24 Duration (minutes) = (volume of fluid bag (eg. 500ml)/ rate) x 60

Answer 160

Rubella- congenital sensorineural deafness and cataracts Toxoplasmosis- Cerebral calcification, Chorioretinitis, Hydrocephalus CMV- Low birth weight, Purpuric skin lesions, congenital sensorineural deafness, Microcephaly, seizures

Answer 161

Primary hypogonadism (Klinefelter's syndrome)- High LH Low testosterone Hypogonadotropic hypogonadism (Kallman's syndrome) Low LH Low testosterone Androgen insensitivity syndrome High LH Normal/high testosterone Testosterone-secreting tumour Low LH High testosterone

Answer 162

Newborn- Otoacoustic emission test: All newborns should be tested as part of the Newborn Hearing Screening Programme Newborn & infants- Auditory Brainstem Response test: May be done if otoacoustic emission test is abnormal 6-9 months- Distraction test: Performed by a health visitor, requires two trained staff

Answer 163

Chondromalacia patellae Softening of the cartilage of the patella Common in teenage girls Characteristically anterior knee pain on walking up and down stairs and rising from prolonged sitting Usually responds to physiotherapy Osgood-Schlatter disease Seen in sporty teenagers Pain, tenderness and swelling over the tibial tubercle Osteochondritis dissecans Pain after exercise Intermittent swelling and locking Patellar subluxation Medial knee pain due to lateral subluxation of the patella Knee may give way Patellar tendonitis More common in athletic teenage boys Chronic anterior knee pain that worsens after running Tender below the patella on examination

Answer 164

Transient synovitis Septic arthritis/osteomyelitis Juvenile idiopathic arthritis Trauma Development dysplasia of the hip Perthes disease Slipped upper femoral epiphysis

Answer 165

Pyloric stenosis Acute appendicitis Intussusception Hirschsprung's disease Meconium ileus Biliary atresia Necrotising enterocolitis

Answer 166

In case 4 year 3

Answer 167

Diphtheria Tetanus Polio Whooping cough (Pertussis) Haemophilus influenzae type B Hepatitis B DTWPHH- don't tango pio when he's h NB- thats the order that they feature in (ie. 3-1 teenage is d t p)

Answer 168

6-1 Rotavirus Meningitis B

Answer 169

6-1 Rotavirus Pneumococcal (PCV) vaccine

Answer 170

6-1 Meningitis B vaccine

Answer 171

Hib/Meningitis C booster MMR Pneumococcal PCV vaccine Meningitis B vaccine

Answer 172

Annual flu vaccine

Answer 173

Diphtheria Tetanus Whooping cough (Pertussis) Polio

Answer 174

HPV vaccine- two jabs are given at 6-24 months apart

Answer 175

3-in-1 teenage booster Meningitis ACWY vaccine

Answer 176

Diphtheria Tetanus Polio

Answer 177

Around 1 in 10 breastfed babies lose more than the 'cut-off' 10% threshold in the first week of life. This should prompt consideration of the above breastfeeding problems. The infant should also be examined to look for any underlying problems. NICE recommends an 'expert' review of feeding if this occurs (e.g. midwife-led breastfeeding clinics) and monitoring of weight until weight gain is satisfactory NB- infants can lose less than 10% of their body weight in the first week of life

Answer 178

Supportive- IV fluids, notify PHE Medical- IV cefotaxime (add IV amoxicillin if younger than 3 months or older than 50 years), IV steroids if older than 3 months, PEP for any contacts of meningococcal disease within 7 days of onset (oral ciprofloxacin) NB- in community IM benzylpenicillin should be used Contraindications to steroids; -Septic shock -Meningococcal septicaemia -Meningitis post-surgery -Immunocompromised

Answer 179

Microcephalic, small eyes Cleft lip/palate Polydactyly Scalp lesions

Answer 180

Micrognathia (small chin) Low-set ears Rocker bottom feet Overlapping of fingers

Answer 181

Learning difficulties Macrocephaly Long face Large ears Macro-orchidism

Answer 182

Webbed neck Pectus excavatum Short stature Pulmonary stenosis

Answer 183

Micrognathia Posterior displacement of the tongue (may result in upper airway obstruction) Cleft palate

Answer 184

Characteristic cry (hence the name) due to larynx and neurological problems Feeding difficulties and poor weight gain Learning difficulties Microcephaly and micrognathism Hypertelorism

Answer 185

Bruises- accidental injury, non-accidental injury eg. physical abuse, coagulopathy (VWD), haematological malignancy, DIC (meningococcal septicaemia (meningitis or sepsis)), ITP, HSP, ED (or other hypermobility syndrome) Fractures- Accidental Injury, Osteogenesis Imperfecta, vitamin D deficiency, ED or other hypermobility syndrome Burns- SSSS, accidental, bullous impetigo Neglect- Failure to thrive, Inadequate hygiene eg severe nappy rash, infestation, Poor development of emotional attachment to child’s caregiver, Delay in development and speech and language, Poor attendance for school and health appointments e.g. immunisations, Failure to supervise eg toddler hit by car while roaming, Unsupervised young children at home- law not clear

Answer 186

Skeletal Survey- full body CT Neurological Imaging- head CT acutely, head MRI once stable

Answer 187

Discuss with team/seniors Refer to children's services/safeguarding officer on the ward Identify any other children who may be at risk at home

Answer 188

Acute symptomatic seizures are a result of acute insults, including hypoglycaemia, hypoxia, electrolyte derangements, infection, and trauma. Focus on any known triggers in the history, think ‘before’, not ‘after’. They may also be recurrent (e.g recurrent febrile seizures).

Answer 189

Infantile spasms (West syndrome)- brief spasms with severe distress afterwards (not to be confused with infantile colic where the child is distressed during the "spasms" (bringing the legs to the chin)) Rolandic epilepsy- on waking, facial involvement Absence epilepsy/seizures Juvenile myoclonic epilepsy- awareness retained, myoclonic episodes Febrile seizures Vasovagal syncope Reflex anoxic seizures Cataplexy- sudden, strong emotion causes loss of tone

Answer 190

A sustained drop through two or more weight/height centiles is unusual and should be investigated. (ie. a child that was once on the 75th percentile is now on the 25th (that's 2 centiles)) Weight or height or BMI is below the 0.4th centile

Answer 191

Overweight is defined as a body mass index (BMI) above the 85th percentile and obese as above the 95th percentile.

Answer 192

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 193

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 194

Features prodrome, e.g. low-grade fever rash: maculopapular, initially on the face before spreading to the whole body, usually fades by the 3-5 day lymphadenopathy: suboccipital and postauricular Complications arthritis thrombocytopaenia encephalitis myocarditis

Answer 195

Also known as fifth disease or 'slapped-cheek syndrome' Caused by parvovirus B19 Lethargy, fever, headache 'Slapped-cheek' rash spreading to proximal arms and extensor surfaces

Answer 196

Heart failure Pan systolic murmur

Answer 197

Ejection systolic murmur Fixed splitting of S2

Answer 198

Compensated Normotensive Tachycardia Tachypnoea Pale/mottled extremities Oliguria Decompensated Hypotension bradycardia acidotic (Kussmaul) blue anuria

Answer 199

Criteria for immediate request for CT scan of the head (children) Loss of consciousness lasting more than 5 minutes (witnessed) Amnesia (antegrade or retrograde) lasting more than 5 minutes Abnormal drowsiness Three or more discrete episodes of vomiting Clinical suspicion of non-accidental injury Post-traumatic seizure but no history of epilepsy GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department Suspicion of open or depressed skull injury or tense fontanelle Any sign of basal skull fracture (haemotympanum, panda' eyes, cerebrospinal fluid leakage from the ear or nose, Battle's sign) Focal neurological deficit If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object)

Answer 200

Congenital inguinal hernia -Should be surgically repaired soon after diagnosis as at risk of incarceration (<6 weeks do in 2 days, <6 months do in 2 weeks, <6 years do in 2 months) Infantile umbilical hernia -The vast majority resolve without intervention before the age of 4-5 years Complications are rare

Answer 201

Intestinal atresia, which can be subdivided into duodenal atresia, jejunal atresia, or ileal atresia is the most common cause Other causes of neonatal bilious vomiting; Malrotation with volvulus (distention, bowle sounds) Meconium ileus Necrotising enterocolitis

Answer 202

Prostaglandin (E2)- keeps DA patent (if associated CHD eg. TGA) Indomethacin (or ibuprofen)- close DA (used in patent DA)

Answer 203

In adults, IV fluids should be given in second or third degree burns that cover 15% body surface area or more. In children, IV fluids are recommended when burns cover 10% body surface area.

Answer 204

20 ml/kg over less than 10 minutes

Answer 205

Newborn resuscitation helps to establish breathing and circulation. 1. Dry baby and maintain temperature 2. Assess tone, respiratory rate, heart rate 3. If gasping or not breathing give 5 inflation breaths (open up lungs) 4. Reassess (chest movements) 5. If the heart rate is not improving and <60bpm start compressions and ventilation breaths at a rate of 3:1

Answer 206

would present with isolated high AFP and normal oestriol, hCG and inhibin A.

Answer 207

Usually occurs on 1st day of life (as opposed to malrotation with volvulus which also causes bilious vomiting in neonates) The diagnosis of intestinal/duodenal atresia is further supported by the presence of the 'double bubble' on the x-ray. NB- double bubble is for duodenal atresia (D for D), jejunal would show an air fluid level

Answer 208

Stress ulcers may occur in the duodenum of burns patients and are more common in children. Tachycardia Hypotensive Haematemesis