Passmed wrong answers Flashcards

Question

What is therapeutic cooling and when is it used?

Answer 1

Therapeutic cooling (otherwise known as therapeutic hypothermia) is the deliberate lowering of a patient’s body temperature. The intention of this is to cool the brain, and subsequently prevent brain damage. The use of therapeutic cooling in the treatment of carefully selected term neonates with moderate to severe hypoxic ischaemic encephalopathy has been recommended as standard care by NICE. It has been shown in studies to decrease mortality and improve the neurological and neurodevelopmental outcomes of treated neonates. Therapeutic cooling remains the only intervention shown to reduce neuronal damage caused by perinatal hypoxia.

Answer 2

Hypoxic perinatal brain injury is caused by a decrease in the amount of oxygen supplied to an infant's brain just prior to, or during the process of, labour. Neonates who survive a hypoxic brain injury can develop HIE. An estimated 10% - 60% of infants with HIE die during the neonatal period, and an estimated 25% of those who survive suffer from long-term neurological impairment including epilepsy, mental retardation or cerebral palsy.

Answer 3

Primary neuronal death occurs at the time of severe ischaemic insult. Secondary neuronal death (resulting in irreversible failure of mitochondrial function) occurs after a period of at least six hours post ischaemic insult. This model of neuronal death suggests there is a latent period between the successful resuscitation and reperfusion post primary neuronal death, and secondary neuronal death. This period of approximately 1-6 hours provides a therapeutic window for neuroprotective intervention. Therapeutic Cooling is thought to influence the extent of secondary neuronal death in a multifactorial manner. Existing studies suggest it is related to: 1. The reduction of cerebral oxygen consumption 2. An increase in haemoglobin oxygen-binding affinity 3. Inhibition of the synthesis, release, and/or reuptake of neurotransmitters and neuromodulators, including glutamate, glycine, GABA, dopamine, norepinephrine, serotonin, and adenosine. 4. The reduction of nitric oxide, and other toxic free radicals.

Answer 4

1. Neonates with a gestational age ≥36 weeks and birth weight >1800g 2. History of acute perinatal event during delivery associated with period of hypoxia, and/or an Apgar score ≤5 at 10 minutes or at least 10 minutes of  positive-pressure ventilation   3. Severe metabolic acidosis on cord gas, or blood gas taken within 1hr of birth 4. Evidence of moderate-severe HIE – demonstrated by onset of seizures, and/or on the basis of clinical assessment of consciousness level, spontaneous activity, posture, tone, primitive reflexes, and autonomic systems. Electroencephalogram (EEG) is useful in determining eligibility (by detection of abnormal neuronal function and/or seizures). Therapeutic cooling should not be offered to: - Moribund infants - Infants with major congenital/genetic abnormalities where aggressive treatment is deemed inappropriate - Infants with evidence of severe head trauma/intracranial bleeding

Answer 5

Treatment must be initiated within 6 hours of birth and continued for a period of 72 hours. Methods of Cooling: 1. Whole-body cooling- placing the infant on a cooling blanket or mattress circulated with coolant fluid. 2. Selective head cooling- circulating cold water in a cap fitted around the head. Whole-body cooling is most often used. It is reported to be easier to set up, less expensive, and provides easier access to perform EEG. Existing studies however suggest that both methods of cooling are appropriate and equivalent in regard to neonatal outcomes. Temperature is continuously monitored throughout the treatment using either a rectal or nasopharyngeal thermometer. Target Temperatures (rectal or oesophageal): - 33°C to 34°C (for whole body cooling) - 34°C to 35°C (for selective head cooling) Following cooling rewarming should be carried out slowly (0.5°C every 1 to 2 hours) over a period of 6-12 hours.

Answer 6

Close surveillance of infants during the cooling process is required given the risk for complications of both HIE, and the process of cooling itself. Serious adverse effects are reported as rare. Existing studies suggest that the benefits of cooling on survival and neurodevelopment outweigh the (usually) short‐term adverse effects. Reported complications include the following: 1. Cardiac and Pulmonary: Arrhythmias (most commonly Sinus Bradycardia, but Ventricular Arrhythmias also reported), Prolonged QT interval, Reduced Cardiac Output and Hypotension, Reduced Surfactant production, Increased Pulmonary vascular resistance (which can lead to Persistent Pulmonary Hypertension) - For these reasons neonates undergoing therapeutic cooling require continuous cardiorespiratory monitoring, and ventilator support during their treatment. 2. Haematological: Anaemia, Coagulopathy secondary to thrombocytopaenia, Leucopaenia - Some neonates require correction of these abnormalities using blood components. Monitoring for signs of sepsis, due to increased susceptibility, are also required. 3. Renal and Liver Toxicity: Renal impairment (association with urinary retention), Impaired liver function (most commonly hyperbilirubinemia) 4. Metabolic: Metabolic and lactic acidosis, Hypokalaemia, Hypoglycaemia, Hypocalcaemia 5. Neurological: Seizures, Raised intracranial pressure - EEG monitoring is important during the cooling process. Sedation is required for patient comfort and to reduce the patient’s metabolic rate to increase the efficacy of the cooling procedure. 6. Skin: Skin breakdown, Local skin haemorrhage, Subcutaneous fat necrosis - The risk of skin complications developing can be reduced by regular skin inspections, and repositioning of neonates undergoing treatment. Indications to stop hypothermia and rewarm the infant include: - Hypotension (unresponsive to inotropes) - Persistent pulmonary hypertension with associated hypoxemia (despite treatment) - Clinically significant coagulopathy (despite treatment) It is uncommon to stop cooling due to complications. Existing studies suggest this occurs in <10% of cases. Rewarming must be carried out slowly to reduce the risk of complications (most commonly metabolic disturbances, and seizures) occurring.

Answer 7

Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction in the platelet count. Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex. It is an example of a type II hypersensitivity reaction. ITP in children is typically more acute than in adults and may follow an infection or vaccination. Features - bruising - petechial or purpuric rash - bleeding is less common and typically presents as epistaxis or gingival bleeding

Answer 8

- full blood count - should demonstrate an 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

Answer 9

usually, no treatment is required ITP resolves in around 80% of children with 6 months, with or without treatment - advice to avoid activities that may result in trauma (e.g. team sports) 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 *platelet transfusions can be used in an emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by the circulating antibodies

Answer 10

BCG vaccination is recommended for babies up to one year of age who: - are born in areas of the UK with high rates of TB - have a parent or grandparent who was born in a country with high rates of TB

Answer 11

Yellow fever vaccination is recommended for travellers over 1-year old arriving from countries with risk of yellow fever transmission, including sub-Saharan Africa and tropical South America.

Answer 12

Hepatitis A vaccine is recommended children over 1-year-old travelling to certain countries including much of Africa, Asia and Central and South America.

Answer 13

General contraindications to immunisation - confirmed anaphylactic reaction to a previous dose of a vaccine containing the same antigens - confirmed anaphylactic reaction to another component contained in the relevant vaccine (e.g. egg protein) Situations where vaccines should be delayed - febrile illness/intercurrent infection Contraindications to live vaccines - pregnancy - immunosuppression Specific vaccines - DTP: vaccination should be deferred in children with an evolving or unstable neurological condition

Answer 14

With macrolides eg erythromycin

Answer 15

Co-amoxiclav

Answer 16

- consider tailored clinical intervention if BMI at 91st centile or above. - consider assessing for comorbidities if BMI at 98th centile or above

Answer 17

By far the most common cause of obesity in childhood is lifestyle factors. Other associations of obesity in children include: Asian children: four times more likely to be obese than white children female children taller children: children with obesity are often above the 50th percentile in height Cause of obesity in children - growth hormone deficiency - hypothyroidism - Down's syndrome - Cushing's syndrome - Prader-Willi syndrome

Answer 18

- orthopaedic problems: slipped upper femoral epiphyses, Blount's disease (a development abnormality of the tibia resulting in bowing of the legs), musculoskeletal pains - psychological consequences: poor self-esteem, bullying - sleep apnoea - benign intracranial hypertension - long-term consequences: increased incidence of type 2 diabetes mellitus, hypertension and ischaemic heart disease

Answer 19

Retinoblastoma is the most common ocular malignancy found in children. The average age of diagnosis is 18 months. Pathophysiology - autosomal dominant - caused by a loss of function of the retinoblastoma tumour suppressor gene on chromosome 13 - around 10% of cases are hereditary

Answer 20

- absence of red-reflex, replaced by a white pupil (leukocoria) - the most common presenting symptom - strabismus - visual problems

Answer 21

- enucleation is not the only option - depending on how advanced the tumour is other options include external beam radiation therapy, chemotherapy and photocoagulation

Answer 22

excellent, with > 90% surviving into adulthood

Answer 23

Neonatal sepsis occurs when a serious bacterial or viral infection in the blood affects babies within the first 28 days of life. Neonatal sepsis is categorised into early-onset (EOS, within 72 hours of birth) and late-onset (LOS, between 7-28 days of life) sepsis, with each category tending to have a distinct group of causes and common presentations.

Answer 24

1. The overall most common causes of neonatal sepsis are group B streptococcus (GBS) and Escherichia coli, accounting for approximately two thirds of neonatal sepsis cases. Early-onset sepsis in the UK is primarily caused by GBS infection (75%)- Infective causes in early-onset sepsis are usually due to transmission of pathogens from the mother to the neonate during delivery Late-onset sepsis usually occurs via the transmission of pathogens from the environment post-delivery, this is normally from contacts such as the parents or healthcare workers - Infective causes are more commonly coagulase-negative staphylococcal species such as Staphylococcus epidermidis, Gram-negative bacteria such as Pseudomonas aeruginosa, Klebsiella and Enterobacter, and fungal species Other less common causes include: - Staphylococcus aureus - Enterococcus - Listeria monocytogenes - Viruses including herpes simplex and enterovirus

Answer 25

- Mother who has had a previous baby with GBS infection, who has current GBS colonisation from prenatal screening, current bacteruria, intrapartum temperature ≥38ºC, membrane rupture ≥18 hours, or current infection throughout pregnancy - Premature (<37 weeks): approximately 85% of neonatal sepsis cases are in premature neonates - Low birth weight (<2.5kg): approximately 80% are low birth weight - Evidence of maternal chorioamnionitis

Answer 26

1. Respiratory distress (85%) - Grunting, Nasal flaring. Use of accessory respiratory muscles, Tachypnoea 2. Tachycardia: common, but non-specific 3. Apnoea (40%) 4. Apparent change in mental status/lethargy 5. Jaundice (35%) 6. Seizures (35%): if cause of sepsis is meningitis 7. Poor/reduced feeding (30%) 8. Abdominal distention (20%) 9. Vomiting (25%) 10. Temperature: not usually a reliable sign as the temperature can vary from being raised, lowered or normal. Term infants are more likely to be febrile. Pre-term infants are more likely to be hypothermic 11. The clinical presentation can vary from very subtle signs of illness to clear septic shock 12. Frequently, the symptoms will be related to the source of infection (e.g. pneumonia + respiratory symptoms, meningitis + neurological symptoms)

Answer 27

1. Blood culture: this will usually establish the diagnosis, as the sensitivity for septicaemia is around 90%. Should usually obtain two blood cultures if possible to distinguish from contamination, however one culture is still sufficient depending on hospital protocol 2. Full blood examination: neonatal sepsis is often associated with abnormal neutrophil counts (both neutrophilia and neutropenia), however in neonates, parameters on full blood examination are usually not always useful for diagnosis, rather may help to exclude healthy neonates 3. C-reactive protein: not useful for diagnosis, but sequential assessment will help to guide management and patient progress with treatment. CRP will usually be raised, and a persistently normal level is likely to exclude neonatal sepsis 4. Blood gases: metabolic acidosis is particularly concerning for neonatal sepsis, particularly a base deficit of ≥10 mmol/L 5. Urine microscopy, culture and sensitivity: rarely positive in EOS, more useful in LOS. Will show signs of infection (e.g. raised leukocytes, positive culture, haematuria, proteinuria) if urinary tract infection is the source of sepsis 6. Lumbar puncture: particularly if there is concern of meningitis as the source of sepsis based on clinical features, however many hospitals will require lumbar puncture as part of a septic screen in any baby below the age of 28 days

Answer 28

1. In neonatal sepsis, the most important part of management to improve outcomes is early identification and treatment 2. The NICE guidelines recommend use of intravenous benzylpenicillin with gentamicin as a first-line regimen for suspected or confirmed neonatal sepsis - The exception to this is if microbiological surveillance data reveal local bacterial resistance patterns, in which case an alternative antibiotic should be considered - CRP should be re-measured 18–24 hours after presentation in babies given antibiotics to monitor ongoing progress and guide duration of therapy - The evidence suggests that antibiotics can be ceased at 48 hours in neonates who have CRP of <10 mg/L and a negative blood culture at presentation and at 48 hours - In all other neonates, the duration of antibiotic treatment will depend on the ongoing investigations and clinical picture, this will involve specialist decision making. Normally, in neonates with culture-proven sepsis, duration will be approximately 10 days 2. Other important management factors to consider include: - Maintaining adequate oxygenation status - Maintaining normal fluid and electrolyte status: severely ill neonates may require volume and/or vasopressor support. Body weight needs to be measured daily for accurate assessment of fluid status - Prevention and/or management of hypoglycaemia - Prevention and/or management of metabolic acidosis

Answer 29

- 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 30

- pregnancy - sexually transmitted infections, recurrent UTIs - sexually precocious behaviour - anal fissure, bruising - reflex anal dilatation - enuresis and encopresis - behavioural problems, self-harm - recurrent symptoms e.g. headaches, abdominal pain

Answer 31

Abnormal development or developmental delay refers to a significant lag in a child's physical, cognitive, behavioural, emotional, or social development, relative to established growth milestones. It is crucial for clinicians to identify and address these delays promptly to improve long-term outcomes.

Answer 32

variant of normal, cerebral palsy and neuromuscular disorders (e.g. Duchenne muscular dystrophy)

Answer 33

The root cause may be genetic disorders, prenatal exposure to toxins/drugs/alcohol, premature birth, nutritional deficiencies or environmental factors

Answer 34

- Autism Spectrum Disorder (ASD): Characterised by impairments in social interaction and communication alongside restricted interests and repetitive behaviours. - Cerebral Palsy: A group of permanent movement disorders appearing in early childhood due to abnormal brain development or damage. - Fragile X Syndrome: A genetic condition causing intellectual disability, behavioural challenges and various physical characteristics. - Down Syndrome: A chromosomal disorder leading to intellectual disability and characteristic facial features. - Fetal Alcohol Spectrum Disorders (FASDs): A range of effects that can occur in an individual exposed to alcohol before birth.

Answer 35

- Clinical Examination: A thorough physical and neurological examination to assess the child's developmental milestones. - Investigations: These may include genetic testing, metabolic screening, neuroimaging studies (MRI or CT scan), and hearing/vision assessments. - Referral for Specialist Assessment: If a specific cause is suspected, referral to a paediatric neurologist, geneticist or developmental paediatrician may be necessary. - Early Intervention Services: Regardless of the underlying cause, all children with developmental delays can benefit from early intervention services such as occupational therapy, speech and language therapy, physiotherapy and educational support.

Answer 36

Hand, foot and mouth disease is a self-limiting condition affecting children. It is caused by the intestinal viruses of the Picornaviridae family (most commonly coxsackie A16 and enterovirus 71). It is very contagious and typically occurs in outbreaks at nursery Clinical features - mild systemic upset: sore throat, fever - oral ulcers - followed later by vesicles on the palms and soles of the feet

Answer 37

- symptomatic treatment only: general advice about hydration and analgesia - reassurance no link to disease in cattle - children do not need to be excluded from school *the HPA recommends that children who are unwell should be kept off school until they feel better *they also advise that you contact them if you suspect that there may be a large outbreak.

Answer 38

Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old. Associations - Beckwith-Wiedemann syndrome - as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation - hemihypertrophy - around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11

Answer 39

- abdominal mass (most common presenting feature) - painless haematuria - flank pain - other features: anorexia, fever - unilateral in 95% of cases - metastases are found in 20% of patients (most commonly lung)

Answer 40

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 41

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

Answer 42

Innocent murmurs heard in children include 1. Ejection murmurs - Due to turbulent blood flow at the outflow tract of the heart 2. 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 3. Still's murmur- Low-pitched sound heard at the lower left sternal edge Characteristics of an innocent ejection murmur include: - soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area - may vary with posture - localised with no radiation - no diastolic component - no thrill - no added sounds (e.g. clicks) - asymptomatic child - no other abnormality

Answer 43

Scarlet fever is a reaction to erythrogenic toxins produced by Group A haemolytic streptococci (usually Streptococcus pyogenes). It is more common in children aged 2 - 6 years with the peak incidence being at 4 years. Scarlet fever is spread via the respiratory route by inhaling or ingesting respiratory droplets or by direct contact with nose and throat discharges, (especially during sneezing and coughing).

Answer 44

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

Answer 45

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 (phenoxymethylpenicillin) - 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

Answer 46

- 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 but may present acutely with life-threatening illness

Answer 47

Biliary atresia is a paediatric condition involving either obliteration or discontinuity within the extrahepatic biliary system, which results in an obstruction in the flow of bile. This results in a neonatal presentation of cholestasis in the first few weeks of life. The pathogenesis of biliary atresia is unclear, however, infectious agents, congenital malformations and retained toxins within the bile are all contributing factors.

Answer 48

Type 1: The proximal ducts are patent, however, the common duct is obliterated Type 2: There is atresia of the cystic duct and cystic structures are found in the porta hepatis Type 3: There is atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia

Answer 49

- Jaundice extending beyond the physiological two weeks - Dark urine and pale stools - Appetite and growth disturbance, however, may be normal in some cases - Hepatomegaly with splenomegaly - Abnormal growth - Cardiac murmurs if associated cardiac abnormalities present

Answer 50

- Serum bilirubin including differentiation into conjugated and total bilirubin: Total bilirubin may be normal, whereas conjugated bilirubin is abnormally high - Liver function tests (LFTs) including serum bile acids and aminotransferases are usually raised but cannot differentiate between biliary atresia and other causes of neonatal cholestasis - Serum alpha 1-antitrypsin: Deficiency may be a cause of neonatal cholestasis - Sweat chloride test: Cystic fibrosis often involves the biliary tract - Ultrasound of the biliary tree and liver: May show distension and tract abnormalities - Percutaneous liver biopsy with intraoperative cholangioscopy

Answer 51

- Surgical intervention is the only definitive treatment for biliary atresia: Intervention may include dissection of the abnormalities into distinct ducts and anastomosis creation. A hepatoportoenterostomy (HPE) can be performed. This is also known as Kasai portoenterostomy and it allows bile drainage. In this procedure, the blocked bile ducts are removed and replaced with a segment of the small intestine. This restores bile flow from the liver to the proximal small bowel. - Medical intervention includes antibiotic coverage and bile acid enhancers following surgery

Answer 52

- Unsuccessful anastomosis formation - Progressive liver disease - Cirrhosis with eventual hepatocellular carcinoma In cases where surgery fails, liver transplantation may be required in the first two years of life. Transplantation is also considered if there are signs of end-stage liver disease, progressive cholestasis, hepatocellular decompensation or the development of severe portal hypertension.

Answer 53

- Not sitting by 8 months (corrected for gestational age) - Not walking by 18 months (corrected for gestational age) - Early asymmetry of hand function (hand preference) before 1 year (corrected for gestational age) - Persistent toe-walking

Answer 54

If a newborn baby has an abnormal hearing test at birth they are offered the auditory brainstem response test

Answer 55

1. Newborn: Otoacoustic emission test All newborns should be tested as part of the Newborn Hearing Screening Programme. A computer-generated click is played through a small earpiece. The presence of a soft echo indicates a healthy cochlea 2 Newborn & infants: Auditory Brainstem Response test May be done if otoacoustic emission test is abnormal 3. 6-9 months: Distraction test Performed by a health visitor, requires two trained staff 4. 18 months - 2.5 years: Recognition of familiar objects Uses familiar objects e.g. teddy, cup. Ask child simple questions - e.g. 'where is the teddy?' 5. > 2.5 years: Performance testing - 6. > 2.5 years: Speech discrimination tests Uses similar-sounding objects e.g. Kendall Toy test, McCormick Toy Test 7. > 3 years: Pure tone audiometry Done at school entry in most areas of the UK As well as the above test there is a questionnaire for parents in the Personal Child Health Records - 'Can your baby hear you?'

Answer 56

to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa

Answer 57

3 months: Quietens to parents voice, Turns towards sound, Squeals 6 months: Double syllables 'adah', 'erleh' 9 months: Says 'mama' and 'dada', Understands 'no' 12 months: Knows and responds to own name 12-15 months: Knows about 2-6 words (Refer at 18 months), Understands simple commands - 'give it to mummy' 2 years: Combine two words, Points to parts of the body 2½ years: Vocabulary of 200 words 3 years: Talks in short sentences (e.g. 3-5 words), Asks 'what' and 'who' questions, Identifies colours, Counts to 10 (little appreciation of numbers though) 4 years: Asks 'why', 'when' and 'how' questions

Answer 58

12 months 3years and 4 months (40 months)

Answer 59

8,12 and 16 weeks 1. Diptheria 2. Tetanus 3. Pertussis (whooping cough) 4. Polio 5. Haemophilus Influenzae type b (Hib) 6. Hepatitis B

Answer 60

8 weeks and 16 weeks Helps to protect against pneumococcal infection which can cause diseases like pneumonia, septicaemia and meningitis

Answer 61

8 weeks and 16 weeks Booster at 1 year

Answer 62

8 weeks. 12 weeks

Answer 63

1. Diptheria 2. Tetanus 3. Whooping cough 4. Polio Given between 3-4 years

Answer 64

1. Tetanus 2. Diptheria 3. Polio Given 13-18 years

Answer 65

Acute lymphoblastic leukaemia (ALL) is the most common malignancy affecting children and accounts for 80% of childhood leukaemias. The peak incidence is at around 2-5 years of age and boys are affected slightly more commonly than girls Features may be divided into those predictable by bone marrow failure: - anaemia: lethargy and pallor - neutropaenia: frequent or severe infections - thrombocytopenia: easy bruising, petechiae And other features - bone pain (secondary to bone marrow infiltration) - splenomegaly - hepatomegaly - fever is present in up to 50% of new cases (representing infection or constitutional symptom) - testicular swelling

Answer 66

1. common ALL (75%), CD10 present, pre-B phenotype 2. T-cell ALL (20%) 3. B-cell ALL (5%)

Answer 67

Pseudomonas aeruginosa is an opportunistic gram-negative bacteria that causes significant morbidity and mortality in patients with cystic fibrosis (CF). This is a clinically important transition stage in the care of a patient with CF, and attempts should be made to eradicate the bacteria if possible because Pseudomonas aeruginosa colonisation can cause a rapid decline in lung function and increased hospitalisations for pneumonia. Oral ciprofloxacin is recommended and is combined with inhaled antibiotics for eradication.

Answer 68

Continuous murmur heard loudest under the left clavicle

Answer 69

ASD's have a fixed split S2 sound due to the increased venous return overloading the right ventricle during inspiration and delaying closure of the pulmonary valve.

Answer 70

VSD are associated with a pansystolic murmur.

Answer 71

Herpesvirus type 6 Generalised pink papular or maculpapular rash which starts on the trunk before spreading to the face and limbs. The high fever (lasts about 3 days) precedes the rash, whivh appears 1-2 weeks later and once the rash appears, the child's temperature returns to normal (ie rash occurs as fever is disappearing)

Answer 72

Transient synovitis is sometimes referred to as irritable hip. 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 is present in a minority of patients - high fever should raise the suspicion of other causes such as septic arthritis - fever is a red flag, indicating the need for urgent specialist assessment - children may be monitored in primary care (with a presumptive diagnosis of transient synovitis) 'If the child is aged 3–9 years, well, afebrile, mobile but limping, and has had the symptoms for less than 72 hours Transient synovitis is self-limiting, requiring only rest and analgesia.

Answer 73

- female sex: 6 times greater risk - breech presentation - positive family history - firstborn children - oligohydramnios - birth weight > 5 kg - congenital calcaneovalgus foot deformity

Answer 74

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 all infants are screened at both the newborn check and also the six-week baby check using the Barlow and Ortolani tests - 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

Answer 75

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

Answer 76

- 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 77

Chronic infection with Burkholderia cepacia

Answer 78

Gastroschisis describes a congenital defect in the anterior abdominal wall just lateral to the umbilical cord. This allows the intestines to extend outside of the baby's body Management - vaginal delivery may be attempted - newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours, whilst waiting the bowel should be protected with cling-film.

Answer 79

In exomphalos (also known as an omphalocoele) 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 (and gradual repair helps to prevent respiratory complications) *if this occurs the sacs is allowed to granulate and epithelialise over the coming weeks/months *this forms a 'shell' *as the infant grows a point will be reached when the sac contents can fit within the abdominal cavity. At this point the shell will be removed and the abdomen closed

Answer 80

1. Bronchodilator therapy - give a beta-2 agonist via a spacer (for a child < 3 years use a close-fitting mask) - give 1 puff every 30-60 seconds up to a maximum of 10 puffs - if symptoms are not controlled repeat beta-2 agonist and refer to hospital 2. Steroid therapy - should be given to all children with an asthma exacerbation - treatment should be given for 3-5 days

Answer 81

1. avoid irritants 2. simple emollients - large quantities should be prescribed (e.g. 250g / week), roughly in a ratio of with topical steroids of 10:1 - 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) 3. topical steroids 4. wet wrapping - large amounts of emollient (and sometimes topical steroids) applied under wet bandages 5. in severe cases, oral ciclosporin may be used

Answer 82

Normal/small testes

Answer 83

1. Gonadotrophin dependent ('central', 'true') - due to premature activation of the hypothalamic-pituitary-gonadal axis - FSH & LH raised 2. Gonadotrophin independent ('pseudo', 'false') - due to excess sex hormones - FSH & LH low In males it is uncommon and usually has an organic cause.

Answer 84

Widening of joints due to an excess of non-mineralised osteoid at the growth plate 'rickety rosary' - swelling at the costochondral joint

Answer 85

Klinefelter's syndrome, a type of primary hypogonadism, is associated with karyotype 47, XXY Usually patients with Kleinfelter syndrome appear as normal males until puberty. At puberty can develop features suggestive of the condition: - Taller height - Wider hips - Gynaecomastia: increased incidence of breast cancer - Weaker muscles - Small, firm testicles - Reduced libido - Shyness - Infertility - Subtle learning difficulties (particularly affecting speech and language) - lack of secondary sexual characteristics - elevated gonadotrophin levels (LH) - Low Testosterone Diagnosis is by chromosomal analysis

Answer 86

Life expectancy is close to normal. Infertility can occasionally be treated with advanced IVF techniques. There is a slight increased risk of: - Breast cancer compared with other males (but still less than females) - Osteoporosis - Diabetes - Anxiety and depression

Answer 87

There is no way to treat the underlying genetic cause of Klinefelter syndrome. Treatment aims to help with the features of the condition: - Testosterone injections improve many of the symptoms - Advanced IVF techniques have the potential to allow fertility - Breast reduction surgery for cosmetic purposes Multidisciplinary team input: - Speech and language therapy to improve speech and language - Occupational therapy to assist in day to day tasks - Physiotherapy to strengthen muscles and joints - Educational support where required for dyslexia and other learning difficulties

Answer 88

Kallman's syndrome is a recognised cause of delayed puberty secondary to hypogonadotrophic hypogonadism. It is usually inherited as an X-linked recessive trait. Kallman's syndrome is thought to be caused by failure of GnRH-secreting neurons to migrate to the hypothalamus. The clue given in many questions is lack of smell (anosmia) in a boy with delayed puberty Features - 'delayed puberty' - hypogonadism, cryptorchidism - anosmia - sex hormone levels are low - LH, FSH levels are inappropriately low/normal - patients are typically of normal or above average height - Cleft lip/palate and visual/hearing defects are also seen in some patients

Answer 89

A deficiency of LH and FSH is the result of abnormal functioning of the hypothalamus or pituitary gland. This could be due to: 1. Previous damage to the hypothalamus or pituitary, for example by radiotherapy or surgery for previous cancer 2. Growth hormone deficiency 3. Hypothyroidism 4. Hyperprolactinaemia (high prolactin) 5. Serious chronic conditions can temporarily delay puberty (e.g. cystic fibrosis or inflammatory bowel disease) 6. Excessive exercise or dieting can delay the onset of menstruation in girls 7. Constitutional delay in growth and development is a temporary delay in growth and puberty without underlying physical pathology 8. Kallman syndrome

Answer 90

Androgen insensitivity syndrome is a condition where cells are unable to respond to androgen hormones due to a lack of androgen receptors. It is an X-linked recessive genetic condition, caused by a mutation in the androgen receptor gene on the X chromosome. Extra androgens are converted into oestrogen, resulting in female secondary sexual characteristics. It was previously known as testicular feminisation syndrome. Patients with androgen insensitivity syndrome are genetically male, with XY sex chromosome. However, the absent response to testosterone and the conversion of additional androgens to oestrogen result in a female phenotype externally. Typical male sexual characteristics do not develop, and patients have normal female external genitalia and breast tissue. Patients have testes in the abdomen or inguinal canal, and absence of a uterus, upper vagina, cervix, fallopian tubes and ovaries. The female internal organs do not develop because the testes produce anti-Müllerian hormone, which prevents males from developing an upper vagina, uterus, cervix and fallopian tubes. The insensitivity to androgens also results in a lack of pubic hair, facial hair and male type muscle development. Patients tend to be slightly taller than the female average. Patients are infertile, and there is an increased risk of testicular cancer unless the testes are removed. There is also a condition called partial androgen insensitivity syndrome, where there the cells have a partial response to androgens. This presents with more ambiguous signs and symptoms, such as a micropenis or clitoromegaly, bifid scrotum, hypospadias and diminished male characteristics. Features - 'primary amenorrhoea' - undescended testes causing groin swellings - breast development may occur as a result of conversion of testosterone to oestradiol - Raised LH, Normal/raised FSH and Testosterone, Raised oestrogen (for a male) Diagnosis - buccal smear or chromosomal analysis to reveal 46XY genotype

Answer 91

- counselling - raise child as female - bilateral orchidectomy (increased risk of testicular cancer due to undescended testes) - oestrogen therapy

Answer 92

Ladd's procedure In malrotation, the caecum remains fixed in the right upper quadrant by peritoneal bands called ladd bands. Ladd's procedure involves the untwisting of the volvulus and the removal of Ladd's bands, resection of necrotic bowel if present and removal of the appendix, to prevent future operations.

Answer 93

Kasai procedure. It involves creating a connection between the liver and the small intestine to allow for bile drainage.

Answer 94

Ramstedt pyloromyotomy is used to manage pyloric stenosis. It consists of a longitudinal incision of the hypertrophic sphincter to split the muscle and allow the passage of food.

Answer 95

Normal term babies often have hypoglycaemia especially in the first 24 hrs of life but without any sequelae, as they can utilise alternate fuels like ketones and lactate. There is no agreed definition of neonatal hypoglycaemia but a figure of < 2.6 mmol/L is used in many guidelines. Transient hypoglycaemia in the first hours after birth is common. Persistent/severe hypoglycaemia may be caused by: - preterm birth (< 37 weeks) - maternal diabetes mellitus - IUGR - hypothermia - neonatal sepsis - inborn errors of metabolism - nesidioblastosis - Beckwith-Wiedemann syndrome

Answer 96

1. may be asymptomatic 2. autonomic (hypoglycaemia → changes in neural sympathetic discharge) - 'jitteriness' - irritable - tachypnoea - pallor 3. neuroglycopenic - poor feeding/sucking - weak cry - drowsy - hypotonia - seizures 4. other features may include - apnoea - hypothermia

Answer 97

Management depends on the severity of the hypoglycaemia and if the newborn is symptomatic 1. asymptomatic - encourage normal feeding (breast or bottle) - monitor blood glucose 2. symptomatic or very low blood glucose - admit to the neonatal unit - intravenous infusion of 10% dextrose

Answer 98

Cow's milk protein intolerance/allergy (CMPI/CMPA) occurs in around 3-6% of all children and typically presents in the first 3 months of life in formula-fed infants, although rarely it is seen in exclusively breastfed infants. 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

Answer 99

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

Answer 100

If the symptoms are severe (e.g. failure to thrive) refer to a paediatrician. 1. 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 - around 10% of infants are also intolerant to soya milk 2. Management if breastfed - continue breastfeeding - eliminate cow's milk protein from maternal diet. Consider prescribing calcium supplements for breastfeeding mothers whose babies have, or are suspected to have, CMPI, to prevent deficiency whilst they exclude dairy from their diet - use eHF milk when breastfeeding stops, until 12 months of age and at least for 6 months

Answer 101

CMPI usually resolves in most children 1. in children with IgE mediated intolerance around 55% will be milk tolerant by the age of 5 years 2. in children with non-IgE mediated intolerance most children will be milk tolerant by the age of 3 years 3. a challenge is often performed in the hospital setting as anaphylaxis can occur.

Answer 102

Characteristic: - Sensorineural deafness - Congenital cataracts - Congenital heart disease (e.g. patent ductus arteriosus) - Glaucoma (heart, eyes and ears are classic rubella triad) Other: - Growth retardation - Hepatosplenomegaly - Purpuric skin lesions - 'Salt and pepper' chorioretinitis - Microphthalmia - Cerebral palsy

Answer 103

Characteristic: - Low birth weight - Purpuric skin lesions - Sensorineural deafness - Microcephaly Other: - Visual impairment - Learning disability - Encephalitis/seizures - Pneumonitis - Hepatosplenomegaly - Anaemia - Jaundice - Cerebral palsy

Answer 104

Characteristic: - Cerebral calcification - Chorioretinitis - Hydrocephalus Other: - Anaemia - Hepatosplenomegaly - Cerebral palsy

Answer 105

If a pregnant woman becomes infected with parvovirus B19 (also known as slapped-cheek disease), consequences to the foetus include hydrops fetalis and sometimes miscarriage or stillbirth. Hydrops fetalis is caused by severe anaemia, in this case, due to a combination of haemolytic anaemia and the virus affecting red blood cell precursors in the infant, and presents with oedema. The oedema is due to high output heart failure. Management is with blood transfusions. Whilst thrombocytopenia may be present in 40%, leading to a petechial rash, severe anaemia and oedema are the presiding features. Hepatomegaly may also be seen, due to its association with haemolytic anaemia, but this is not common.

Answer 106

more serious if bronchopulmonary dysplasia (e.g. Premature), congenital heart disease or cystic fibrosis

Answer 107

Turner's syndrome Haemophilia is a X-linked recessive disorder and would hence be expected only to occur in males. As patients with Turner's syndrome only have one X chromosome however, they may develop X-linked recessive conditions

Answer 108

1. Jaundice in the first 24 hours - Jaundice in the first 24 hours is always pathological. - Causes of jaundice in the first 24 hrs *rhesus haemolytic disease *ABO haemolytic disease *hereditary spherocytosis *glucose-6-phosphodehydrogenase 2. Jaundice in the neonate from 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. - It is more commonly seen in breastfed babies 3. Jaundice after 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 *jaundice is more common in breastfed babies mechanism is not fully understood but thought to be due to high concentrations of beta-glucuronidase → increase in intestinal absorption of unconjugated bilirubin *prematurity *due to immature liver function *increased risk of kernicterus *congenital infections e.g. CMV, toxoplasmosis

Answer 109

- RNA paramyxovirus - one of the most infectious known viruses - spread by aerosol transmission - infective from prodrome until 4 days after rash starts - incubation period = 10-14 days

Answer 110

Features 1. prodromal phase - irritable - conjunctivitis - fever 2. Koplik spots - typically develop before the rash - white spots ('grain of salt') on the buccal mucosa 3. Rash - starts behind ears then to the whole body - discrete maculopapular rash becoming blotchy & confluent - desquamation that typically spares the palms and soles may occur after a week 4. diarrhoea occurs in around 10% of patients

Answer 111

Investigations - IgM antibodies can be detected within a few days of rash onset Management - mainly supportive - admission may be considered in immunosuppressed or pregnant patients - notifiable disease → inform public health

Answer 112

- 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 113

- if a child not immunized against measles comes into contact with measles then MMR should be offered (vaccine-induced measles antibody develops more rapidly than that following natural infection) - this should be given within 72 hours

Answer 114

Undescended testis occurs in around 2-3% of term male infants, but is much more common if the baby is preterm. Around 25% of cases are bilateral.' Management 1. 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 2. Bilateral undescended testes - Should be reviewed by a senior paediatrician within 24hours as the child may need urgent endocrine or genetic investigation

Answer 115

- Seriously ill - Poor urine flow - Abdominal or bladder mass - Raised creatinine - Septicaemia - Failure to respond to treatment with suitable antibiotics within 48 hours - Infection with non-E. coli organisms.

Answer 116

Oral phenoxymethylpenicillin (azithromycin in penicillin allergy)

Answer 117

100-120bpm

Answer 118

1. Antibiotics < 3 months: IV amoxicillin (or ampicillin) -to cover for listeria monocytogenes + IV cefotaxime > 3 months: IV cefotaxime (or ceftriaxone) 2. Steroids NICE advise against giving corticosteroids in children younger than 3 months dexamethsone should be considered if the lumbar puncture reveals any of the following: - frankly purulent CSF - CSF white blood cell count greater than 1000/microlitre - raised CSF white blood cell count with protein concentration greater than 1 g/litre - bacteria on Gram stain 3. Fluids treat any shock, e.g. with colloid 4. Cerebral monitoring mechanical ventilation if respiratory impairment 5. Public health notification and antibiotic prophylaxis of contacts ciprofloxacin is now preferred over rifampicin

Answer 119

A) Patau syndrome (trisomy 13) Microcephalic, small eyes, Cleft lip/palate, Polydactyly, Scalp lesions B) Edward's syndrome (trisomy 18) Micrognathia, Low-set ears, Rocker bottom feet, Overlapping of fingers C) Fragile X Learning difficulties, Macrocephaly, Long face, Large ears, Macro-orchidism D) Noonan syndrome Webbed neck, Pectus excavatum, Short stature, Pulmonary stenosis E) Pierre-Robin syndrome Micrognathia, Posterior displacement of the tongue (may result in upper airway obstruction), Cleft palate F) Prader-Willi syndrome Hypotonia, Hypogonadism, Obesity G) William's syndrome Short stature, Learning difficulties, Friendly, extrovert personality, Transient neonatal hypercalcaemia, Supravalvular aortic stenosis H) Cri du chat syndrome (chromosome 5p deletion syndrome) Characteristic cry (hence the name) due to larynx and neurological problems, Feeding difficulties and poor weight gain, Learning difficulties, Microcephaly and micrognathism, Hypertelorism

Answer 120

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 121

jittery movements increased muscle tone hyperreflexia convulsions drowsiness or coma

Answer 122

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 (seen in 50%) 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 123

Following birth, the first step is to dry the baby maintain temperature and start the clock. Following this you assess tone breathing and heart rate. If gasping or not breathing, you open the airway to give 5 inflation breaths. You then reassess for an increase in the heart rate. If there is no increase in the heart rate you ensure the inflation breaths you are giving are adequate by checking chest movement. If the chest is not moving you assume the inflation breaths are inadequate and recheck head position, consider 2-person airway control and other manoeuvers and repeat inflation breaths then look for a response. If the chest is moving but the heart rate is still undetectable or less than 60 beats per minute you start chest compressions at a ratio of 3 compressions to 1 inflation breath (3:1). You reassess heart rate every 30 seconds, and if it is still undetectable or very slow, you consider IV access and drugs.

Answer 124

The Apgar score is used to assess the health of a newborn baby. NICE recommend that it is assessed at 1, and 5 minutes of age. If the score is low then it is again repeated at 10 minutes. Stands for Appearance (colour), Pulse (heart rate), Grimace (reflex irritability), Activity (muscle tone), Respiratory effort. 1. Pulse *2 - > 100 *1 - < 100 *0 - Absent 2. Respiratory effort *2 - Strong, crying *1 - Weak, irregular *0 - Nil 3. Colour *2 - Pink *1 - Body pink, extremities blue *0 - Blue all over 4. Muscle tone *2 - Active movement *1 - Limb flexion *0 - Flaccid 5. Reflex irritability *2 - Cries on stimulation/sneezes, coughs *2 - Grimace *0 - Nil A score of 0-3 is very low score, between 4-6 is moderate low and between 7 - 10 means the baby is in a good state

Answer 125

early changes include widening of joint space, later changes include decreased femoral head size/flattening

Answer 126

osteoarthritis premature fusion of the growth plates (could present with an antalgic gait with apparent leg shortening and referred knee pain)

Answer 127

Slipped capital femoral epiphysis is rare hip condition seen in children, classically seen in obese boys. It is also is known as slipped upper femoral epiphysis. Basics - typically age group is 10-15 years - More common in obese children and boys - Displacement of the femoral head epiphysis postero-inferiorly - May present acutely following trauma or more commonly with chronic, persistent symptoms Features - hip, groin, medial thigh or knee pain - loss of internal rotation of the leg in flexion - bilateral slip in 20% of cases

Answer 128

- short palpebral fissure - thin vermillion border/hypoplastic upper lip - smooth/absent filtrum - learning difficulties - microcephaly - growth retardation - epicanthic folds - cardiac malformations

Answer 129

- it is an oral, live attenuated vaccine - 2 doses are required, the first at 8 weeks, the second at 12 weeks - 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 Other points - the vaccine is around 85-90% effective and is predicted to decrease hospitalisation by 70% - offers long-term protection against rotavirus

Answer 130

The outcome or prognosis of a patient with CDH is largely dependent on 2 factors (1) Liver position (2) Lung-to-head ratio - If the liver has herniated into the chest, the disease is more severe and there is lower chance of survival. - The lung-to-head ratio is a numeric estimate of the size of the foetal lungs, dependent on the amount of lung visible. A ratio >1.0 reflects a better outcome.

Answer 131

Congenital diaphragmatic hernia (CDH) occurs in around 1 in 2,000 newborns. It is characterised by the herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm. This can result in pulmonary hypoplasia and hypertension which causes respiratory distress shortly after birth. Pathophysiology - usually represents a failure of the pleuroperitoneal canal to close completely The most common type of CDH is a left-sided posterolateral Bochdalek hernia which accounts for around 85% of cases. 13% of cases happen on the right and 2% bilaterally. Only around 50% of newborns with CDH survive despite modern medical intervention.

Answer 132

- respiratory rate above 60 per minute - the presence of grunting - heart rate below 100 or above 160 beats/minute - capillary refill time above 3 seconds - temperature of 38°C or above, or 37.5°C on 2 occasions 30 minutes apart - oxygen saturation below 95% - presence of central cyanosis

Answer 133

- To keep the femoral head within the acetabulum: cast, braces - If less than 6 years: observation (+/- physiotherapy and serial x-rays) - Older: surgical management with moderate results - Operate on severe deformities

Answer 134

3 times more common in males Down's syndrome

Answer 135

- 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 136

In 2012 there was an outbreak of whooping cough (pertussis) which resulted in the death of 14 newborn children. As a temporary measure, a vaccination programme was introduced in 2012 for pregnant women. This has successfully reduced the number of cases of whooping cough (the vaccine is thought to be more than 90% effective in preventing newborns developing whooping cough). It was however decided in 2014 to extend the whooping cough vaccination programme for pregnant women. This decision was taken as there was a 'great deal of uncertainty' about the timing of future outbreaks. Women who are between 16-32 weeks pregnant will be offered the vaccine. There is no single whooping cough vaccine so women are given the Boostrix IPX which has pertussis, diphtheria and tetanus)

Answer 137

Surfactant deficient lung disease (SDLD, also known as respiratory distress syndrome and previously as hyaline membrane disease) is a condition seen in premature infants. It is caused by insufficient surfactant production and structural immaturity of the lungs. Deficiency of surfactant results in an increased alveolar surface tension, thereby resulting in decreased compliance and increased work of breathing. The risk of SDLD decreases with gestation 50% of infants born at 26-28 weeks 25% of infants born at 30-31 weeks Other risk factors for SDLD include - male sex - diabetic mothers - Caesarean section - second born of premature twins

Answer 138

Clinical features are those common to respiratory distress in the newborn, i.e. tachypnoea, intercostal recession, expiratory grunting and cyanosis Chest x-ray characteristically shows 'ground-glass' appearance with an indistinct heart border

Answer 139

- prevention during pregnancy: maternal corticosteroids to induce fetal lung maturation - oxygen - assisted ventilation - exogenous surfactant given via endotracheal tube

Answer 140

Necrotising enterocolitis is one of the leading causes of death among premature infants. Initial symptoms can include feeding intolerance, abdominal distension and bloody stools, which can quickly progress to abdominal discolouration, perforation and peritonitis.

Answer 141

- dilated bowel loops (often asymmetrical in distribution) - bowel wall oedema - pneumatosis intestinalis (intramural gas) - portal venous gas - pneumoperitoneum resulting from perforation air both inside and outside of the bowel wall (Rigler sign) - air outlining the falciform ligament (football sign)

Answer 142

Atlantoaxial instability is a complication of Down Syndrome that increases the risk of sudden neck dislocation. It is strongly advised people with Down syndrome who participate in sports that may carry an increased risk of neck dislocation (e.g. trampolining, gymnastics, boxing, diving, rugby and horse riding) are screened for atlantoaxial instability.

Answer 143

Serial rectal irrigation/washouts should be performed before surgery to help prevent enterocolitis.

Answer 144

Urgent hospital assessment. Because they are at higher risk of septic arthritis and child maltreatment.

Answer 145

plain X-ray of both hips (AP and frog-leg views) The diagnosis can be confirmed and graded if Klein's line (drawn along the superior edge of the femoral neck) intersects less of the femoral head. Since SUFE is bilateral in 20% of cases, both hips should be imaged to exclude involvement of the other hip, even if there are no suggestive symptoms currently.

Answer 146

high-flow oxygen nebulised adrenaline

Answer 147

NICE CKS advises that for localised non-bullous impetigo, you can consider prescribing hydrogen peroxide 1% cream (apply two or three times daily for 5 days) for people who are not systemically unwell or at a high risk of complications. Only if this is 'unsuitable', should you consider topical antibiotics (first line is fusidic acid 2%, with mupirocin 2% as second line if fusidic acid resistance is suspected or confirmed). A child with impetigo should be excluded from school until the lesions are crusted and healed or 48 hours after commencing antibiotic treatment

Answer 148

In infants, due to their short necks, the carotid pulse is often difficult to palpate and the femoral pulse is preferred. According to basic life support (BLS) guidelines, infants (children under 1 year) should have femoral and brachial pulse assessed. The carotid pulse may only be used in children (over 12 months old)

Answer 149

isolated thrombocytopenia

Answer 150

1. usually, no treatment is required - ITP resolves in around 80% of children with 6 months, with or without treatment 2. advice to avoid activities that may result in trauma (e.g. team sports) 3. 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 - platelet transfusions can be used in an emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by the circulating antibodies

Answer 151

bilateral enlargement = gonadotrophin release from intracranial lesion unilateral enlargement = gonadal tumour Normal/ small testes = adrenal cause (tumour or adrenal hyperplasia)

Answer 152

A non-retractable foreskin The British Association of Paediatric Urologists states that if the issue is a non-retractile foreskin and/or ballooning during micturition in a child under two, an expectant approach should be taken in case this is physiological phimosis which will resolve in time. Forcible retraction can result in scar formation so should be avoided. Personal hygiene is important. If the child is over 2 years of age and has recurrent balanoposthitis or urinary tract infection then treatment can be considered.

Answer 153

Neonates who are cyanotic are given 100% oxygen for 15 minutes and an ABG is performed. The test is positive if the pO2 is <15kPa, which indicates a cyanotic heart defect. A negative test suggests respiratory or central cause.

Answer 154

cyanosis tachypnoea loud single S2 prominent right ventricular impulse 'egg-on-side' appearance on chest x-ray

Answer 155

Colour: Normal Activity: * Responds normally to social cues * Content/smiles * Stays awake or awakens quickly * Strong normal cry/not crying Circulation and hydration: * Normal skin and eyes * Moist mucous membranes Management: Child can be managed at home with appropriate care advice, including when to seek further help

Answer 156

Amber = intermediate risk Colour: * Pallor reported by parent/carer Activity: * Not responding normally to social cues * No smile * Wakes only with prolonged stimulation * Decreased activity Respiratory: * Nasal flaring * Tachypnoea: respiratory rate >50 breaths/minute, age 6-12 months; >40 breaths/minute, age >12 months * Oxygen saturation <=95% in air * Crackles in the chest Circulation and hydration: * Tachycardia: >160 beats/minute, age <12 months >150 beats/minute, age 12-24 months >140 beats/minute, age 2-5 years * Capillary refill time >=3 seconds * Dry mucous membranes * Poor feeding in infants * Reduced urine output Other: * Age 3-6 months, temperature >=39ºC * Fever for >=5 days * Rigors * Swelling of a limb or joint * Non-weight bearing limb/not using an extremity Management: - Provide parents with a safety net or refer to a paediatric specialist for further assessment - A safety net includes verbal or written information on warning symptoms and how further healthcare can be accessed, a follow-up appointment, liaison with other healthcare professionals, e.g. out-of-hours providers, for further follow-up

Answer 157

Colour: * Pale/mottled/ashen/blue Activity: * No response to social cues * Appears ill to a healthcare professional * Does not wake or if roused does not stay awake * Weak, high-pitched or continuous cry Respiratory: * Grunting * Tachypnoea: respiratory rate >60 breaths/minute * Moderate or severe chest indrawing Circulation and hydration: * Reduced skin turgor Other: * Age <3 months, temperature >=38°C * Non-blanching rash * Bulging fontanelle * Neck stiffness * Status epilepticus * Focal neurological signs * Focal seizures Management: Refer child urgently to a paediatric specialist

Answer 158

Bicuspid aortic valve (Ejection systolic murmur) Coarctation of the aorta

Answer 159

William's syndrome, also known as Williams-Beuren syndrome, is a rare genetic disorder characterised by supravalvular aortic stenosis (SVAS), intellectual disability, distinctive facial features (a broad forehead, puffiness around the eyes, a flat bridge of the nose, full cheeks, and a small chin. Many affected people have dental problems such as teeth that are small, widely spaced, crooked, or missing), and a unique personality profile (friendly/extrovert). SVAS in William's syndrome results from an elastin gene deletion on chromosome 7q11.23. This condition leads to narrowing of the aorta above the level of the aortic valve, causing increased resistance to blood flow out of the heart and potentially leading to left ventricular hypertrophy.

Answer 160

Macrogol (movicol/laxido/cosmocol) Polyethylene glycol Lactulose All are stool softeners Macrogol used first line in children (or third line addition of lactulose) and second line in adults

Answer 161

Bisacodyl (dulcolax) Senna Sodium picosulfate Second line in children if Movicol doesn't work, third line addition in adults if other laxatives fail

Answer 162

Laryngomalacia (typically presents at 4 weeks of age)

Answer 163

Males - first sign is testicular growth at around 12 years of age (range = 10-15 years), testicular volume > 4 ml indicates onset of puberty (Tanner stage 2) - Increase in penis length follows (Tanner stage 3) - Both above may coincide with development of pubic hair - Height spurt occurs mid puberty (Tanner stage 3-4) - Development of axillary hair (Tanner stage 4-5) 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 164

Ophthalmia neonatorum simply means infection of the newborn eye. Responsible organisms include Chlamydia trachomatis Neisseria gonorrhoeae Suspected ophthalmia neonatorum should be referred for same-day ophthalmology/paediatric assessment.

Answer 165

blood culture, full blood count, c-reactive protein, urine testing for urinary tract infections, stool culture is diarrhoea present, and chest x-ray if there are respiratory signs.

Answer 166

A cephalohaematoma is seen as a swelling on the newborns head. It typically develops several hours after delivery and is due to bleeding between the periosteum and skull - therefore they do not cross over the suture lines of the skull. The most common site affected is the parietal region. Jaundice may develop as a complication. A cephalohaematoma can take up to 3 months to resolve.

Answer 167

Caput succedaneum is an extraperiosteal collection of blood therefore can cross over the suture lines and can be present at birth. It forms over the vertex and resolves within days

Answer 168

Simple: <15 min Generalised No recurrence in 24 hours Complete recovery in an hour Complex: 15-30 minutes Focal seizure May have repeat seizures in 24 hours

Answer 169

- 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 - if recurrent febrile convulsions occur then benzodiazepine rescue medication may be considered *this should only be started on the advice of a specialist (e.g. a paediatrician) *rectal diazepam or buccal midazolam may be used

Answer 170

3 months Reaches for object Holds rattle briefly if given to hand Visually alert, particularly human faces Fixes and follows to 180 degrees 6 months Holds in palmar grasp Pass objects from one hand to another Visually insatiable, looking around in every direction 9 months Points with finger Early pincer 12 months Good pincer grip Bangs toys together

Answer 171

15 months - Tower of 2 18 months - Tower of 3 2 years - Tower of 6 3 years - Tower of 9 18 months - Circular scribble 2 years - Copies vertical line 3 years - Copies circle 4 years - Copies cross 5 years - Copies square and triangle

Answer 172

Trinucleotide repeat disorders are genetic conditions caused by an abnormal number of repeats (expansions) of a repetitive sequence of three nucleotides. These expansions are unstable and may enlarge which may lead to an earlier age of onset in successive generations - a phenomenon known as anticipation*. In most cases, an increase in the severity of symptoms is also noted - Fragile X (CGG) - Huntington's (CAG) - myotonic dystrophy (CTG) - Friedreich's ataxia* (GAA) - spinocerebellar ataxia - spinobulbar muscular atrophy - dentatorubral pallidoluysian atrophy *Friedreich's ataxia is unusual in not demonstrating anticipation

Answer 173

ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually cause a murmur)

Answer 174

Alpha-thalassaemia is due to a deficiency of alpha chains in haemoglobin 2 separate alpha-globulin genes are located on each chromosome 16 (4 alleles in total) Clinical severity depends on the number of alpha globulin alleles affected: - If 1 or 2 alpha globulin alleles are affected then the blood picture would be hypochromic and microcytic, but the Hb level would be typically normal - If 3 alpha globulin alleles are affected results in a hypochromic microcytic anaemia with splenomegaly. This is known as Hb H disease - If all 4 alpha globulin alleles are affected (i.e. homozygote) then death in utero (hydrops fetalis, Bart's hydrops)

Answer 175

Hib/Men C MMR Pneumococcal Men B

Answer 176

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 duodenal atresia Hirschsprung's disease

Answer 177

Roseola infantum (also known as exanthem subitum, occasionally sixth disease) is a common disease of infancy caused by the human herpes virus 6 (HHV6). It has an incubation period of 5-15 days and typically affects children aged 6 months to 2 years. Features - high fever: lasting a few days, followed 1-2 weeks 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 178

Referral points - doesn't smile at 10 weeks - cannot sit unsupported at 12 months - cannot walk at 18 months

Answer 179

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 180

Radionuclide scan using dimercaptosuccinic acid (DMSA). DMSA renal scintigraphy is considered the gold standard for detecting renal parenchymal defects, such as scarring due to vesicoureteric reflux. It involves administering a radiopharmaceutical agent, dimercaptosuccinic acid (DMSA), which is taken up by healthy renal tubular cells and allows for imaging of the kidneys. Areas that do not take up DMSA represent scars or non-functioning kidney tissue.

Answer 181

Webbed neck, pulmonary stenosis, ptosis and short stature.

Answer 182

Per nasal swab (Bordetella pertussis adhered strongly to ciliated epithelial cells high up in nasopharynx which may not be adequately samples with throat swabs?

Answer 183

(most commonly coxsackie A16 and enterovirus 71).

Answer 184

- obesity - nasal problems: polyps, deviated septum, hypertrophic nasal turbinates - recurrent tonsillitis - Down's syndrome - hypothyroidism

Answer 185

- regular (at least twice daily) chest physiotherapy and postural drainage. Parents are usually taught to do this. Deep breathing exercises are also useful - high calorie diet, including high fat intake - patients with CF should try to minimise contact with each other to prevent cross infection with Burkholderia cepacia complex and Pseudomonas aeruginosa - vitamin supplementation - pancreatic enzyme supplements taken with meals - lung transplantion *chronic infection with Burkholderia cepacia is an important CF-specific contraindication to lung transplantation

Answer 186

- is used to treat cystic fibrosis patients who are homozygous for the delta F508 mutation - lumacaftor increases the number of CFTR proteins that are transported to the cell surface - ivacaftor is a potentiator of CFTR that is already at the cell surface, increasing the probability that the defective channel will be open and allow chloride ions to pass through the channel pore

Answer 187

Meckel's diverticulum is a congenital diverticulum of the small intestine. It is a remnant of the omphalomesenteric duct (also called the vitellointestinal duct) and contains ectopic ileal, gastric or pancreatic mucosa. . Rule of 2s occurs in 2% of the population is 2 feet from the ileocaecal valve is 2 inches long

Answer 188

Intussusception

Answer 189

Fragile X syndrome is a trinucleotide repeat disorder. Fragile X syndrome is caused by a mutation in the FMR1 (fragile X mental retardation 1) gene on the X chromosome. The FMR1 gene codes for the fragile X mental retardation protein, which plays a role in cognitive development in the brain. Features in males - learning difficulties - large low set ears, long thin face, high arched palate - macroorchidism - hypotonia - Hypermobile joints (particularly in the hands) - ADHD - autism is more common - mitral valve prolapse Features in females (who have one fragile chromosome and one normal X chromosome) range from normal to mild

Answer 190

Diagnosis - can be made antenatally by chorionic villus sampling or amniocentesis - analysis of the number of CGG repeats using restriction endonuclease digestion and Southern blot analysis Management There is no cure for the condition. Management is supportive and involves treating the symptoms. This involves the multidisciplinary team to support the learning disability, manage autism and ADHD and treat seizures if they occur. Life expectancy is similar to the general population depending on associated disabilities and complications.

Answer 191

Transient tachypnoea of the newborn (TTN) is 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

Answer 192

Testicular torsion - acutely severe testicular pain with associated nausea and vomiting, swelling of testis with overlying erythema, cremaster reflex absent on affected side, elevation of testicle worsens pain Epididymitis - Urinary symptoms, cremaster reflex present, elevation of testes reduces pain

Answer 193

Infantile spasms, or West syndrome, is a type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more common in male infants. They are often associated with a serious underlying condition and carry a poor prognosis Features - characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms - this lasts only 1-2 seconds but may be repeated up to 50 times - progressive mental handicap

Answer 194

Investigation the EEG shows hypsarrhythmia in two-thirds of infants CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis) Management poor prognosis vigabatrin is now considered first-line therapy ACTH is also used

Answer 195

Macrolide if onset of cough is within 21 days

Answer 196

SpO2 < 92% (unlike in adults, SpO2 < 92% may be consistent with a 'severe' attack in children) PEF 33-50% best or predicted Too breathless to talk or feed Heart rate >125 (>5 years) >140 (1-5 years) Respiratory rate >30 breaths/min (>5 years) >40 (1-5 years) Use of accessory neck muscles

Answer 197

SpO2 <92% PEF <33% best or predicted Silent chest Poor respiratory effort - normal pco2 Agitation Altered consciousness Cyanosis

Answer 198

Benign rolandic epilepsy is a form of childhood epilepsy that typically occurs between the age of 4 and 12 years. Features - seizures characteristically occur at night - seizures are typically partial (e.g. paraesthesia affecting the face) but secondary generalisation may occur (i.e. parents may only report tonic-clonic movements) - the child is otherwise normal EEG characteristically shows centrotemporal spikes The prognosis is excellent, with seizures stopping by adolescence

Answer 199

Intraventricular haemorrhage is a haemorrhage that occurs into the ventricular system of the brain. It is relatively rare in adult surgical practice and when it does occur, it is typically associated with severe head injuries. In premature neonates it may occur spontaneously. The blood may clot and occlude CSF flow, hydrocephalus may result. In neonatal practice the vast majority of IVH occur in the first 72 hours after birth, the aetiology is not well understood and it is suggested to occur as a result of birth trauma combined with cellular hypoxia, together the with the delicate neonatal CNS. Treatment Is largely supportive, therapies such as intraventricular thrombolysis and prophylactic CSF drainage have been trialled and not demonstrated to show benefit. Hydrocephalus and rising ICP is an indication for shunting.

Answer 200

Complications of undescended testis - infertility - torsion - testicular cancer - psychological

Answer 201

it displaces bilirubin from albumin binding sites, resulting in higher levels of bilirubin that accumulate in the tissues.

Answer 202

maternal antiepileptic use increases risk

Answer 203

Plagiocephaly parallelogram shaped head the incidence of plagiocephaly has increased over the past decade. This may be due to the success of the 'Back to Sleep' campaign. The vast majority improve by age 3-5 due to the adoption of a more upright posture. Helmets are not usually recommended as there was no significant difference between groups in a randomised controlled trial. Turning the cot around may help the child look the other way and take the pressure off the one side. Other simple methods include giving the baby time on their tummy during the day, supervised supported sitting during the day, and moving toys/ mobiles around in the cot to change the focus of attention. Ensure all advice is in line with prevention of SIDS.

Answer 204

Duchenne muscular dystrophy is 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

Answer 205

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 206

- 1% foetuses affected if mother has primary infection in weeks 3-28 due to deactivation in utero - Features: skin scarring, eye defects (small eyes, cataracts or chorioretinitis), neurological defects (reduced IQ, abnormal sphincter function, microcephaly)

Answer 207

Maternal syphilis infection: - Rhinitis, saddle shaped nose, deafness (sensorineural hearing loss) and Hutchinson’s incisors - Hepatosplenomegaly, lymphadenopathy, anaemia, jaundice

Answer 208

Pulmonary hypoplasia is a term used for newborn infants with underdeveloped lungs Causes include oligohydramnios congenital diaphragmatic hernia

Answer 209

return 5 days from onset of rash

Answer 210

Return 24 hours after commencing antibiotics

Answer 211

Return 2 days after commencing antibiotics (or 21 days from onset of symptoms if no abx)

Answer 212

Return 4 days from onset of rash

Answer 213

Return 5 days from onset of swollen glands

Answer 214

No exclusion

Answer 215

6 weeks - Smiles (Refer at 10 weeks) 3 months - Laughs, Enjoys friendly handling 6 months - Not shy 9 months - Shy, Takes everything to mouth, Plays 'peek-a-boo' 12 months - Waves 'bye-bye' Plays 'pat-a-cake' 18 months - Plays contentedly alone 2 years - Plays near others, not with them 4 years - Plays with other children

Answer 216

1. infants less than 3 months old should be referred immediately to a paediatrician 2. children aged more than 3 months old with an 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. children aged more than 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. Parents should be asked to bring the children back if they remain unwell after 24-48 hours 4. antibiotic prophylaxis is not given after the first UTI but should be considered with recurrent UTIs

Answer 217

- short stature - diabetes mellitus - delayed puberty - rectal prolapse (due to bulky stools) - nasal polyps - male infertility, female subfertility

Answer 218

Atopic eczema, also known as atopic dermatitis, typically affects the face and trunk in infants. This distribution is due to the fact that these areas are more exposed to environmental allergens and irritants. The cheeks, forehead, and extensor surfaces of the limbs are common sites. The nappy area is usually spared because it is covered and protected from these factors.

Answer 219

Intubation and ventilation Insertion of a nasogastric tube with the aim of keeping air out of the gut

Answer 220

Head lice (also known as pediculosis capitis or 'nits') is a common condition in children caused by the parasitic insect Pediculus capitis, which lives on and among the hair of the scalp of humans. Head lice are small insects that live only on humans, they feed on our blood. Eggs are grey or brown and about the size of a pinhead. The eggs are glued to the hair, close to the scalp and hatch in 7 to 10 days. Nits are the empty egg shells and are white and shiny. They are found further along the hair shaft as they grow out. Head lice are spread by direct head-to-head contact and therefore tend to be more common in children because they play closely together. They cannot jump, fly or swim! When newly infected, cases have no symptoms but itching and scratching on the scalp occurs 2 to 3 weeks after infection. There is no incubation period.

Answer 221

Diagnosis - fine-toothed combing of wet or dry hair Management - 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 School exclusion is not advised for children with head lice

Answer 222

> 24 hours old

Answer 223

NICE recommend immediate referral (usually by 999 ambulance) if they have any of the following: - apnoea (observed or reported) - child looks seriously unwell to a healthcare professional - severe respiratory distress, for example grunting, marked chest recession, or a respiratory rate of over - 70 breaths/minute - central cyanosis - persistent oxygen saturation of less than 92% when breathing air. NICE recommend that clinicians 'consider' referring to hospital if any of the following apply: - a respiratory rate of over 60 breaths/minute - difficulty with breastfeeding or inadequate oral fluid intake (50-75% of usual volume 'taking account of risk factors and using clinical judgement') - clinical dehydration.

Answer 224

1. antenatal (80%): e.g. cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV) 2. intrapartum (10%): birth asphyxia/trauma 3. postnatal (10%): intraventricular haemorrhage, meningitis, head-trauma

Answer 225

Possible manifestations include: - abnormal tone early infancy - delayed motor milestones - abnormal gait - feeding difficulties. Children with cerebral palsy often have associated non-motor problems such as: - learning difficulties (60%) - epilepsy (30%) - squints (30%) - hearing impairment (20%)

Answer 226

1. spastic (70%) subtypes include hemiplegia, diplegia or quadriplegia increased tone resulting from damage to upper motor neurons 2. dyskinetic caused by damage to the basal ganglia and the substantia nigra athetoid movements and oro-motor problems 3. ataxic caused by damage to the cerebellum with typical cerebellar signs 4. mixed

Answer 227

- as with any child with a chronic condition a multidisciplinary approach is needed - treatments for spasticity include oral diazepam, oral and intrathecal baclofen, botulinum toxin type A, orthopaedic surgery and selective dorsal rhizotomy - anticonvulsants, analgesia as required

Answer 228

Tetralogy of fallot

Answer 229

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

Answer 230

Nephrotic syndrome is classically defined as a triad of - proteinuria (> 1 g/m^2 per 24 hours) - hypoalbuminaemia (< 25 g/l) - oedema In children the peak incidence is between 2 and 5 years of age. Around 80% of cases in children are due to a condition called minimal change glomerulonephritis. The condition generally carries a good prognosis with around 90% of cases responding to high-dose oral steroids. Other features include hyperlipidaemia, a hypercoagulable state (due to loss of antithrombin III) and a predisposition to infection (due to loss of immunoglobulins)

Answer 231

Prader-Willi syndrome is an example of genetic imprinting where the phenotype depends on whether the deletion occurs on a gene inherited from the mother or father: - Prader-Willi syndrome if gene deleted from father - Angelman syndrome if gene deleted from mother

Answer 232

Prader-Willi syndrome is associated with the absence of the active Prader-Willi gene on the long arm of chromosome 15. This may be due to: - microdeletion of paternal 15q11-13 (70% of cases) - maternal uniparental disomy of chromosome 15 Features - hypotonia during infancy - dysmorphic features - short stature - hypogonadism and infertility - learning difficulties - childhood obesity - behavioural problems in adolescence

Answer 233

Streptococcus pneumoniae

Answer 234

- Group B Streptococcus: usually acquired from the mother at birth. More common in low birth weight babies and following prolonged rupture of the membranes - E. coli and other Gram -ve organisms - Listeria monocytogenes

Answer 235

Neisseria meningitidis (meningococcus) Streptococcus pneumoniae (pneumococcus) Haemophilus influenzae

Answer 236

Neisseria meningitidis (meningococcus) Streptococcus pneumoniae (pneumococcus)

Answer 237

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. It's most common in teenagers who participate in sports involving running or jumping but typically presents with localised tenderness over tibial tuberosity and visible enlargement of it rather than joint swelling or locking. Also, Osgood-Schlatter disease causes discomfort on activity but rarely causes pain at rest unlike osteochondritis dissecans. Management is supportive

Answer 238

Lactose intolerance Transient lactose intolerance is a common complication of viral gastroenteritis. Removal of lactose from the diet for a few months followed by a gradual reintroduction usually resolves the problem.

Answer 239

This is because insulin, which is increased in diabetic mothers, inhibits surfactant production and maturation of the fetal lungs.

Answer 240

Neonatal blood spot screening (previously called the Guthrie test or 'heel-prick test') is performed at 5-9 days of life The following conditions are currently screened for: - congenital hypothyroidism - cystic fibrosis - sickle cell disease - phenylketonuria - medium chain acyl-CoA dehydrogenase deficiency (MCADD) - maple syrup urine disease (MSUD) - isovaleric acidaemia (IVA) - glutaric aciduria type 1 (GA1) - homocystinuria (pyridoxine unresponsive) (HCU)

Answer 241

- abdominal pain mimicking appendicitis - rectal bleeding * Meckel's diverticulum is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages of 1 and 2 years - intestinal obstruction *secondary to an omphalomesenteric band (most commonly), volvulus and intussusception

Answer 242

if the child is haemodynamically stable with less severe or intermittent bleeding then a 'Meckel's scan' should be considered uses 99m technetium pertechnetate, which has an affinity for gastric mucosa mesenteric arteriography may also be used in more severe cases e.g. transfusion is required

Answer 243

removal if narrow neck or symptomatic options are between wedge excision or formal small bowel resection and anastomosis

Answer 244

Achondroplasia is an autosomal dominant disorder associated with short stature. It is caused by a mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene. This results in abnormal cartilage giving rise to: - short limbs (rhizomelia) with shortened fingers (brachydactyly) - large head with frontal bossing and narrow foramen magnum - midface hypoplasia with a flattened nasal bridge - 'trident' hands - lumbar lordosis In most cases (approximately 70%) it occurs as a sporadic mutation. The main risk factor is advancing parental age at the time of conception. Once present it is typically inherited in an autosomal dominant fashion. (With homozygous (two dominant alleles) achondroplasia children unfortunately don't live past the first few months of life)

Answer 245

- advise regarding position during feeds - 30 degree head-up - infants should sleep on their backs as per standard guidance to reduce the risk of cot death - ensure infant is not being overfed (as per their weight) and consider a trial of smaller and more frequent feeds - a trial of thickened formula (for example, containing rice starch, cornstarch, locust bean gum or carob bean gum) for bottle fed babies - a trial of alginate therapy e.g. Gaviscon for breastfed babies. Alginates should not be used at the same time as thickening agents - NICE do not recommend a proton pump inhibitor (PPI) to treat overt regurgitation in infants and children occurring as an isolated symptom. A trial of one of these agents should be considered if 1 or more of the following apply: - unexplained feeding difficulties (for example, refusing feeds, gagging or choking) - distressed behaviour - faltering growth

Answer 246

Meconium aspiration syndrome refers to respiratory distress in the newborn as a result of meconium in the trachea. (cyanosis, tachypnoea, increased W.O.B) It occurs in the immediate neonatal period. It is more common in post-term deliveries, with rates of up to 44% reported in babies born after 42 weeks. It causes respiratory distress, which can be severe. Higher rates occur where there is a history of maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse. CXR shows patchy infiltrations and atelectasis

Answer 247

1. Newly-diagnosed asthma - Short-acting beta agonist (SABA) 2. Not controlled on previous step OR Newly-diagnosed asthma with symptoms >= 3 / week or night-time waking - SABA + an 8-week trial of paediatric MODERATE-dose inhaled corticosteroid (ICS) *After 8-weeks stop the ICS and monitor the child's symptoms: **if symptoms did not resolve during the trial period, review whether an alternative diagnosis is likely **if symptoms resolved then reoccurred within 4 weeks of stopping ICS treatment, restart the ICS at a paediatric low dose as first-line maintenance therapy **if symptoms resolved but reoccurred beyond 4 weeks after stopping ICS treatment, repeat the 8‑week trial of a paediatric moderate dose of ICS 3. SABA + paediatric low-dose ICS + leukotriene receptor antagonist (LTRA) 4. Stop the LTRA and refer to an paediatric asthma specialist

Answer 248

1. Newly-diagnosed asthma - Short-acting beta agonist (SABA) 2. Not controlled on previous step OR Newly-diagnosed asthma with symptoms >= 3 / week or night-time waking - SABA + paediatric low-dose inhaled corticosteroid (ICS) 3. SABA + paediatric low-dose ICS + leukotriene receptor antagonist (LTRA) 4. SABA + paediatric low-dose ICS + long-acting beta agonist (LABA) (In contrast to the adult guidance, NICE recommend stopping the LTRA at this point if it hasn't helped) 5. SABA + switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a paediatric low-dose ICS 6. SABA + paediatric MODERATE-dose ICS MART (OR consider changing back to a fixed-dose of a moderate-dose ICS and a separate LABA) 7. SABA + one of the following options: - increase ICS to paediatric high-dose, either as part of a fixed-dose regime or as a MART - a trial of an additional drug (for example theophylline) - seeking advice from a healthcare professional with expertise in asthma

Answer 249

- putting the baby to sleep prone: the relative risk or odds ratio varies from 3.5 - 9.3. If not accustomed to prone sleeping (i.e. the baby usually sleeps on their back) the odds ratio increases to 8.7-45.4 - parental smoking: studies suggest this increases the risk up to 5 fold - prematurity: 4-fold increased risk - bed sharing: odds ratio 5.1 - hyperthermia (e.g. over-wrapping) or head covering (e.g. blanket accidentally moves)

Answer 250

- 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 - there is now thought to be little role for oral prednisolone in children who do not require hospital treatment

Answer 251

This condition is characterised by a fragment of bone in the knee joint that becomes detached due to a lack of blood supply. The fragment and its overlying cartilage can then move around inside the joint, causing symptoms such as pain, swelling, and locking. It's most common in adolescents and young adults, particularly those who are active or participate in sports.

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