Paediatrics (Quesmed) Flashcards

Question

What are the symptoms of Asthma in children?

Answer 1

Cough, Breathlessness, Wheeze and Chest Tightness Widespread Wheeze heard on Auscultation of the Chest during an Acute Episode 4 Investigations - Serial Peak Flow Readings when both symptomatic and asymptomatic- as airflow obstruction is reversible - Those with high probability of Asthma can be started on SABA - Spirometry should be performed where possible - If Unclear, FeNO tests may be needed

Answer 2

Spacer preferred in children 1) Inhaled SABA PRN and CONSIDER monitored Inhalation of Very Low to Low dosed Inhaled ICS 2) Add Very Low dosed ICS (or LTRA if <5 years old) 3) Very Low dose ICS AND (<5 years old= add LTRA, >5 years old= add LTRA or LABA) (so both this time) ----Add-on therapies- - No response to LABA- stop LABA and increase ICS to Low Dose - If some benefit from LABA but not enough, increase ICS to Low Dose - If benefit from LABA and Low Dose ICS but inadequate, Consider Trial of LTRA High-dose Therapies- - Increase ICS to Medium Dose - Addition of Theophylline (or other 4th Drug) - Refer to specialist care If the above is still not enough, use Daily Steroid Tablet and maintain Medium-dose ICS. Consider Other treatments to minimise steroid use OFFER INFLUENZA VACCINATION TO PATIENTS ON ICS as they are IMMUNOCOMPROMISED

Answer 3

May be Asymptomatic Ejection-Systolic Murmur on Auscultation and Fixed Split Second Heart Sound Loudest on Left-Sternal Edge and is a Flow Murmur through the Pulmonary Valve May present with Heart Failure

Answer 4

Diagnosed via Echocardiogram Management depends on the severity of the lesion. Most are managed Conservatively but some may require Surgery. they are kept under Surveillance through routine Echocardiograms 2 Complications- - Heart Failure- Occurs in Patient's 20s or 30s - Paradoxical Embolism

Answer 5

Conservative- Behavioural Techniques and Extra Support at School Medical- Stimulant medication like Methylphenidate increases activity of Frontal Lobe. If Methylphenidate does not help, then switch to Atomexitine MAIN SIDE EFFECT of Methylphenidate= Growth Suppression due to Appetite Suppression

Answer 6

In its most severe form: - Children with Autism are not able to understand that other people have thoughts and feelings and prefer to play alone and avoid eye contact - Children with Autism have SPEECH AND LANGUAGE DELAY, Monotonous Tones of Voice with Limited Expression and problems using Pronouns. They also interpret speech literally and have problems with the use of Idioms - In terms of behaviour, they have Narrow Interests, Ritualistic Behaviours that rely on heavy routine and Stereotyped Movements (Rocking, Flapping Hand Movements)) ASDs are associated with Learning Difficulties !!! About 25% of children with ASD may also have Seizures !!! Down Syndrome is commonly associated with ASD

Answer 7

MDT management needed with extra support to the family Applied Behavioural Analysis can help- encouraging positive behaviours and discouraging negative behaviours <10% of children with ASD can live independently as adults

Answer 8

It is a rare condition that presents similar to a VIRAL CROUP but the child would have a HIGH FEVER and RAPIDLY PROGRESSIVE AIRWAY OBSTRUCTION with COPIOUS THICK AIRWAY SECRETIONS It is most commonly caused by Staphylococcus Aureus, typically following an Upper Respiratory Tract Infection It is managed via IV Antibiotics in Severe Cases and May require Intubation If Croup with Fever (>38.5C) and does not resolve following medication, THINK BACTERIAL TRACHEITIS

Answer 9

The bile ducts of an infant are fibrosed and destroyed which leads to hyperbilirubinaemia, liver failure with HEPATOMEGALY and death It presents as prolonged obstructive conjugated jaundice (>14 days) and biliary obstruction- dark urine and white stool Diagnosis- - Raised CONJUGATED bilirubin and DERANGED LFTs - Hepatic Scintigraphy highlights the liver but has poor excretion into the bowel as the bile ducts connecting the liver to the bowel have been destroyed - Abdominal ultrasound reveals Echogenic Fibrosis - Definitive diagnosis is confirmed with Cholangiography which fails to show normal architecture of the biliary tree Complications - Liver Disease and Hepatocellular Carcinoma

Answer 10

Surgery- Hepatoportoenterostomy (Kasai Procesure)

Answer 11

Persistent headaches that are worse in the morning Signs of raised ICP Seizure in an older child with NO past history of seizure and NO fever

Answer 12

Astrocytoma (most common) - Pilocystic astrocytoma (doesnt cross midline) - Glioblastoma multiforme (crosses midline- more aggressive) Medullablastoma (second most common- highly aggressive) Meningioma (tumours of arachnoid cells in meninges) Craniopharyngioma (pituitary- can lead to homonymous hemianopia) Ependymomas (tumours of the cells of the ventricular system- lateral etc.)

Answer 13

Diagnosis- MRI or CT Management- Depends on tumor size and staging- Chemotherapy, Radiotherapy and Surgery

Answer 14

Embryological remnant from development of branchial arches- painful cyst in front of sternocleidomastoid (Posterior to sternocleidomastoid and hyperechoic= Lymphatic Malformation) (Moves on swallowing= Thyroglossal Cyst) Management- Conservatively or Surgery

Answer 15

Mothers- - Reduction in risk of diabetes - Reduction in risk of breast cancer - Reduction in risk of ovarian cancer Children- - Protection against infection - Protection against eczema - Protection against Sudden Infant Death Syndrome

Answer 16

HIV mothers should REFRAIN from breastfeeding Hepatitis B mothers do not need to refrain, provided the child is immunised

Answer 17

Sore Nipples Blocked Ducts Mastitis/ Abscess- may need ORAL ANTIBIOTICS and DRAINAGE Thrush and Breastfeeding/ DUCTAL CANDIDIASIS Insufficient Milk

Answer 18

Mother taking amiodarone Neonatal Galactosaemia Mother has HIV Mother has active HSV Mother has TB infection (IBUPROFEN is SAFE)

Answer 19

Benign causes of SEIZURES in children due to Temporary Lack of Blood Flow. This is because of the VAGUS NERVE BEING OVERACTIVE Precipitant- sudden pain or vomiting Child recovers quickly once oxygen is given so management is just REASSURANCE

Answer 20

Young children cry so much, they hold their breath and faint- may turn blue and jerk limbs Management- Just reassurance, they'll be fine

Answer 21

Chest infection- winter epidemic at 1-12 month olds Caused by Respiratory Syncytial Virus

Answer 22

1-12 month olds Cough, Laboured Breathing, Wheeze, Tachypnoea, Nasal Flaring, FEVER (High-grade in Pneumonia) (Breathing Hard and Fast- so hard you're using Big Nostrils) NASAL FLARING or RR>70 is a RED FLAG- may need O2 NON PRODUCTIVE COUGH (productive in Pneumonia) May also be FINE CRACKLES (Focal in Pneumonia)

Answer 23

Pavilizumab Prophylaxis if at risk- CONGENITAL HEART DEFECTS/ CYSTIC FIBROSIS Oxygen Therapy RIBAVIRIN Supportive Care

Answer 24

Hyponatraemia

Answer 25

- Bronchiolitis Obliterans Narrowing of airways due to Chronic Inflammation which causes Scar Tissue Caused by ADENOVIRUS, not RSV, OR transplants

Answer 26

CXRs may often appear normal so CT/ Lung Biopsy needed - FEV1 is decreased (16-21%) Management- - No cure - Immunosuppressants like Cyclosporin, Mycophenolate Mofetil, Tacrolimus and Prednisone can treat it POST-TRANSPLANT

Answer 27

Pressure of top of Infant's skull against the Dilating Cervix during Labour Presents as Subcutaneous Serosanguineous Fluid Collection superficial to cranium but below the skin. It CROSSES SUTURE LINES (like a blister when your foot has been rubbing against the show lol) Management- resolves on its own

Answer 28

Ciprofloxacin (causes Joint Problems) Nitrofurantoin (G6PD Deficiency) Teicoplanin Clindamycin (antibiotic associated colitis) Co-trimoxazole Most antibiotics cause loose motions in the baby or tummy aches- they don't need treatment

Answer 29

Ciprofloxacin (Joint Problems) Nitrofurantoin (G6PD Deficiency) Teicoplanin Clindamycin (Antibiotic-associated Colitis) Co-trimoxazole Most antibiotics cause loose motion or tummy aches in the baby- they don't need treatment for this

Answer 30

Aspirin (causes Reye's Syndrome) Codeine Dihydrocodeine is the preferred Opiate Analgesic for stronger painkillers but observe baby for drowsiness

Answer 31

1st hour to 6 months of life- no other foods

Answer 32

Permanent movement issues due to CNS damage It does NOT get worse though (non-progressive)

Answer 33

Before Birth- - Hypoxic Ischaemic Encephalopathy (suspect if PROLONGED DELIVERY) - Infection After Birth- - Meningitis - Haemorrhage - Trauma - Medication Toxicity - Kernicterus (high amount of bilirubin enters baby's brain) (wouldn't be due to breastmilk jaundice though) Idiopathic

Answer 34

Spastic (90% of patients with CP have spastic features)- - damage to PYRAMIDAL PATHWAYS - Increased TONE and REFLEXES, Clasp Knife, Flexed hip and elbow - may be MONOPLEGIC, DIPLEGIC, HEMIPLEGIC - Scissor Walking- due to Periventricular Lesion Dyskinetic/ Athetoid (this is usually due to KERNICTERUS)- - Damage to Basal Ganglia - Choreiform Movements (involuntary movements) - Signs of Parkinsonism Ataxic- - Damage to CEREBELLAR PATHWAYS - Uncoordinated movements (lack of balance and coordination) - Signs of cerebellar lesions

Answer 35

Specialist and MDT input to deal with complications- - Physiotherapy, Occupational therapy, Speech and Language therapy and Dietician input due to swallowing difficulties Medical management- - BACLOFEN for the spasms - Botox Injections for Contractures Surgical Management- - Orthopaedic Surgery for MSK deformities - General Surgery for PEG feeding tube

Answer 36

Raised, Red Itchy Spots on the face or chest that spread to the rest of the body They then become small Fluid-filled Blisters that then Crust up Conservative management as it is Self-Limiting - Simple measures are to keep fingernails short and wear long clothing to avoid scratching

Answer 37

Over 5- - V5= Orientated to Place or Person - V4= Confused - V3= Inappropriate Words - V2= Incomprehensible Sounds - V1= No Response Under 5- - V5= Alert, Babbles, Coos, or Sentences to Normal Abilities - V4= Less than normal ability - V3= Cries to Pain - V2= Moans to Pain - V1= No Response

Answer 38

It is when the Aorta Narrows (usually location is just before DUCTUS ARTERIOSUS) It is associated with TURNER's Syndrome May be asymptomatic OR - Systolic Murmur- heard LOUDEST BETWEEN SHOULDER BLADES - Radio-femoral and maybe Radio-radial Delay - Hypertension - Heart Failure - If Coarctation is severe, the infant has Shock once the Ductus Arteriosus closes

Answer 39

- Monitored via Echocardiography and Antihypertensives to control the BP - Angioplasty and Stent Insertion may be needed as Intervention - If they have Shock, give them Prostaglandins to keep the Ductus Arteriosus open

Answer 40

Coarctation of Aorta= Cerebral Strokes due to Cerebral Aneurysms and Hypertension Atrial-Septal Defects= Ischaemic Stroke (due to Paradoxical Embolism)

Answer 41

T-cell mediated autoimmune inflammation of the small bowel The sensitivity to Prolamin results in VILLOUS ATROPHY and MALABSORPTION

Answer 42

HLA-DQ2 gene and other autoimmune conditions T1DM It presents at INFANCY and at 50-60 YEARS OLD (Crohn's presents at Adolescence) (Cystic Fibrosis has high Trypsinogen, also look for Resp Infections and Meconium Ileus) (Cow's Milk Allergy= Rash over Face, Reflux, Colicky pain)

Answer 43

Abdominal pain Abdominal Distension Nausea and Vomiting Diarrhoea Steatorrhoea (if severe) (Imagine tummy is full on diarrhoea and vomiting and so full that its ballooning so distension) Systemic Symptoms- - Fatigue and Weight Loss (due to Low Absorption) - Pallor (ANAEMIA) - Short Stature and Wasted Buttocks (if severe) (MALNUTRITION) - VITAMIN DEFICIENCY (like Bruising due to Vitamin K Deficiency) Dermatological (if severe)= Dermatitis Herpetiformis (itchy lesions over bum and extensors (arms, legs, trunk))

Answer 44

ANTIBIOTICS= - Anti-TTG IgA is First Line (check their IgA level as well as they may be IgA Deficient- if so then measure Anti-TTG IgG instead) - If this doesn't work as well (Weakly Positive) then Anti-Endomyseal Antibody as well NICE do not recommend HLA-DQ2 if non specialist or Anti-Gliadin OTHER TESTS= GOLD STANDARD- OGD and SMALL BOWEL BIOPSY= refer if Positive serological test or Negative with High Suspicion Stool Culture- Exclude Infection Histology shows= VILLOUS ATROPHY, CRYPT HYPERPLASIA and INTRAEPIPHELIAL LYMPHOCYTES (cos lymphocytes are breaking villi down and making the crypts bigger) BLOODS= = FBC- Microcytic Anaemia cos of Iron Deficiency, Normocytic Anaemia cos of Chronic Inflammation or Macrocytic Anaemia due to Folate Deficiency = U&Es and Bone Profile (Vitamin D absorption may be affected) = LFTs (Albumin may be low due to Malabsorption) = Iron, B12, Folate (Anaemia stuff)

Answer 45

Life-long Gluten Free Diet and monitor them to make sure they are following it

Answer 46

Anaemia (due to B12, Iron or Folate Deficiency) Hyposplenism (and therefore susceptibility to organisms)- AUTOIMMUNE HYPOSPLENISM OSTEOPOROSIS (so DEXA scan) Enteropathy-associated T Cell Lymphoma (rare type of Non-Hodgkin's)- because of the Intraepithelial T Lymphocytes

Answer 47

Physical aggression, Destructive Behaviour and Stealing in under 18's Prognosis is generally poor- - 50%= Antisocial Personality Disorder - 50%= Substance Misuse - 40%= Become Young offenders Oppositional Defiant Disorder= Anger and Temper, but no Aggression, No Destruction and No Stealing

Answer 48

Genetic Defect in one of the enzymes involved in the synthesis of hormones in the adrenal cortex Signs - Ambiguous Genitalia - Virilisation (female has male features) - HYPOTENSION due to DEHYDRATION and VOMITING - Precocious (Early) Puberty (so these ambiguous changes are more evident)

Answer 49

Salt Wasting CAH - 21-hydroxylase deficiency - Leads to Cortisol and Aldosterone deficiency - This leads to High levels of ACTH Production- this leads to Adrenal Hyperplasia - This leads to Androgen Steroids production- Testosterone and DHEA - Low Aldosterone may not lead to symptoms at birth as baby has Mineralocortoid supply from placenta - Few weeks after birth though- Large amounts of Sodium are lost and Potassium is retained. Leads to Blood Volume Loss and HYPOTENSION. the high amounts of Potassium leads to Metabolic Acidosis and Vomiting and therefore DEHYDRATION Virilising CAH - Residual 21-hydroxylase activity may be retained so Aldosterone levels are normal - Exposure to high levels of Testosterone leads to female fetuses being VIRILISED- leading to Ambiguous Genitalia (same with BOTH TYPES of CAH btw) - In males, no changes like this but can cause Early puberty

Answer 50

Intravenous Saline (if Salt-Wasting) and give Hydrocortisone to replace low Glucocorticoids and Mineralocorticoids Lifelong Treatment with deficient hormones (Glucocorticoids and Mineralocorticoids) Virilised Females- need Surgery

Answer 51

No coordination between Atria and Ventricles- so P waves are dissociated from QRS complexes Causes- most commonly associated with maternal SYSTEMIC LUPUS ERYTHEMATOSUS. Linked to Maternal Anti-Ro and Anti-La antibodies Presentation- May range from being Asymptomatic to Severe Heart Failure. - Neonates may be Bradycardic or Circulatory Shock MOST CHILDREN- no symptoms for first few years of life and then Syncope and Pre-Syncope

Answer 52

Pacemaker if Symptomatic

Answer 53

LEVOTHYROXINE is FIRST LINE Side effects- Osteoporosis and Cardiac Arrhythmias (Weak Bones and Buzzed Heart) AUTOIMMUNE THYROIDITIS

Answer 54

Hypotonia (FLOPPY) Macroglossia (Large Tongue) UMBILICAL HERNIA Reduced Feeding Constipation Remember Floppy and Umbilical Hernia

Answer 55

Child defecates less than 3 times a week or has difficulty passing stool- may also be overflow diarrhoea - Most common Cause is diet - LOW FIBRE diet - Dislike of using toilet - Pain on passing stool - not recognising sensation of needing to pass stool

Answer 56

Child defecates less than 3 times a week or has difficulty passing stool- may also be overflow diarrhoea - Most common Cause is diet - LOW FIBRE diet - Dislike of using toilet - Pain on passing stool - Not recognising sensation of needing to pass stool

Answer 57

Diagnosis- - History and palpation of hardened faeces on abdominal examination Management- - Movicol Disimpaction Regimen - Then Maintenance Movicol, alongside a High Fibre Diet and advice about encouraging Good Toileting Habits

Answer 58

The distal colon of the baby is not innervated properly so the AGANGLIONIC colon is shrunken and not able to distend properly- this causes a back pressure of stool trapped in the proximal colon AT BIRTH maybe-- - Delay in passing MECONIUM (>2 days and no first poop) - Distended abdomen - Forceful evacuation of meconium after Digital Rectal Examination POOR RESPONSE to MOVICOL REGIMENS may occur and POOR WEIGHT GAIN

Answer 59

Diagnosis- Rectal Suction Biopsy Management- Removal of Aganglionic Colon and Healthy Bowel is pulled through

Answer 60

Constipation- Hard stools Hirchsprungs- Explosive release of liquid faecal matter

Answer 61

AKA Laryngotracheobronchitis Caused by: - Parainfluenza- MAIN cause - ADENOVIRUS, INFLUENZA, RSV Usually 6 months to 6 years

Answer 62

Barking, seal cough (apparently leads to Productive) Stridor (ADMIT IF THERE IS A STRIDOR AT REST !!!!!!!!!!!!!!!!!!!!) FEVER Coryza Management - MILD- Dexamethasone - SEVERE- Oxygen and Nebulised Budesonide. (Supplemental Oxygen is MAINSTAY for Bronchiolitis) If significant concerns about airway- Nebulised Adrenaline Make sure they do NOT CRY as this can worsen the Croup

Answer 63

Congenital Heart Disease that results in a Right to Left Shunt 1) Transposition of the great arteries (Pulmonary Artery and Aorta have swapped their insertion) 2) Pulmonary and Tricuspid Atresia (so the right side of the heart is a Dead end) 3) Tetraology of Fallot (Pulmonary Stenosis and Large Ventricular Septal Defect shunting at the Ventricular Level)- also OVERRIDING AORTA and RIGHT VENTRICULAR HYPERTROPHY

Answer 64

Usually antenatally during scans - Presentation occurs within first few weeks of life with babies being VISIBLY CYANOTIC - ECHOCARDIOGRAPHY can help characterise the anatomy and the heart function Management- - Surgical Correction or Heart transplant - Prostaglandin E can be given to delay Ductus Arteriosus closing - AVOID Ibuprofen as it causes the Ductus Arteriosus to close faster

Answer 65

Babies can maintain Normal Saturations if Large VSD (so NO MURMUR) and Patent Ductus Arteriosus (whose murmurs are not heard until pressure builds up) to compensate the Transposition of the Great Arteries So when the ductus does close, saturations can fall due to Right-to-Left Shunting. Giving Oxygen does not make a difference as Pulmonary Circulation is already saturated well

Answer 66

Autosomal Recessive Disorder- causes LUNG INFECTIONS and LIMITS ability to breathe Cause- Mutations affecting CFTR protein (most common one affects the DELTA Protein)

Answer 67

Respiratory System- - Thick Mucus in lungs - !!!!!!!!!!!!SINUSITIS and NASAL POLYPS is also very common. Patients usually have Pseudonomas colonisation by 20 years old Digestive System- - Reduced Pancreatic Enzyme Secretion inhibits the absorption of Fat which causes Steatorrhoea - This leads to the deficiency of Fat-Soluble Enzymes (Vitamin ADEK) - Poor Absorption of fat leads to Poor Weight Gain - Damage to Pancreas puts patients at risk of Diabetes Reproductive System- - Seminiferous Tubes get blocked- most men with Cystic Fibrosis are unable to conceive naturally - Fertility is also lower in women due to Thicker Cervical Membrane

Answer 68

Neonates- - Meconium Ileus due to the Thick Mucus making the Meconium Thicker - Treated with GASTROGRAFFIN ENEMA Infants- (like NaCli with breathing issues) - Salty Sweat - Faltering Growth - Recurrent Chest Infections Toddlers- (NaCli with digestive issues) - Faltering Growth - Recurrent Chest Infections - MALABSORPTION Older Children- - Faltering Growth - Recurrent Chest Infections - MALABSORPTION - DELAYED onset Puberty

Answer 69

Diagnosis- - Sweat Test (Definitive and better)- High Chloride - Heel Prick Test- High Trypsinogen Management- (Clear the Chest, give them ABD, support the Pancreas and VACCINATE) - MDT management - Chest Physiotherapy to clear Mucus and prevent Pneumonia - Medical management- Antibiotics, Bronchodilators and Secretion-thinners (Dornase Alfa) - Pancreatic Enzyme Replacement (CREON) and Vitamin supplementation (ADEK) - INFLUENZA and PNEUMOCOCCAL Vaccinations - LAST RESORT= Lung Transplant

Answer 70

ACRODERMATITIS ENTEROPATHICA - Rash - Hairloss - Diarrhoea

Answer 71

Pellagra (the 3 D's) - Dermatitis - Diarrhoea - Dementia

Answer 72

No puberty by 14 in boys and 13 in girls Most common cause is Constitutional Delay (confirmed with WRIST XRAY- Epiphyseal Plates are late to fuse) Rarer Causes - Low gonadotrophin- Hypothyroidism, Systemic Disease (Cystic Fibrosis or Coeliac Disease), Pituitary Disorders - High gonadotrophin- Chromosomal Disorders (Klinefelters and Turners), Congenital Adrenal Hyperplasia, Hypogonadism (like after Chemotherapy)

Answer 73

Sad most of the day Change in sleep Change in physical activity Change in Weight and Appetite Fatigue Concentration affected Anhedonia Guilt/ Worthlessness Suicidality

Answer 74

1) Neurological (Parkinson's, Dementia, Multiple Sclerosis) 2) Endocrine Disorders (Thyroid/ Hypo or Hyperadrenalism) 3) Drugs (Steroids, Isoretinoin, Alcohol, Beta blockers, Benzodiazepines, Methyldopa) 4) Chronic Conditions (Diabetes, Obstructive Sleep Apnoea, Long-standing infections like Mononucleosis) 5) Neoplasms and Cancers 6) Other Psychiatric Conditions- Bipolar Disorder, Schizophrenia, Dementia, Seasonal Affective Disorder)

Answer 75

1st Line- Group CBT/ Low Intensity Psychological Interventions 2nd Line- Pharmacological Therapy (SSRIs) / CBT Antidepressants should be continued for at least 6 MONTHS after Remission, gradually reducing over a 4 WEEK PERIOD IF Actively Suicidal= then URGENT REFERRAL, so SCREEN for this asap If SEVERE= Start with Pharmacological and add on Psychological Therapy - ECT for Life-threatening (but can cause memory loss and risk of damage to teeth as it is an Induced Seizure)

Answer 76

Mild- watchful waiting and healthy habits Moderate to severe- refer to CAMHS - First Line- Psychological Therapy - FLUOXETINE- first line in children - Admission if risk of self harm and suicide

Answer 77

Ball of the Femur and Acetabulum of the Pelvis do not articulate properly 5 Fs for Risk Factors - Firstborn - Female - Fanny First (Breech Presentation) - Fluid (Oligohydramnios) - Family History

Answer 78

Screening- 2 Manouevres - Barlow (To find out if there is Posterior Dislocation) - Ortalani (Relocation of the Dislocated hip on Abduction of Hip) Diagnosis- Hip Ultrasound Management- - Mild= sorts itself out in first few months (if no noticeable symptoms) - Severe= PAVLIK Harness or Surgery GALLEAZI TEST= if there is shortening, this helps figure out if it is the Tibia or the Femur

Answer 79

Transient Synovitis - Treated with NSAIDs and Bed Rest

Answer 80

Posseting- Small amounts of white fluid is common after feeds in infants (this is normal) Testicular Torsion- If testicular pain Brain Tumour- If neurological signs Infection- More FORCEFUL vomits may be signs of infection, so ENT EXAMINATION is recommended for all vomiting children Whooping Cough- Vomiting after long periods of Coughing (especially if UNVACCINATED)- Bordetella Pertussis GORD- Large volume regurgitation can be GORD- manage with Positioning and Burping advice Pyloric Stenosis- PROJECTILE vomiting in Young Boys whilst Palpable OLIVE Mass is felt at 6-8 weeks, after feeding- Surgery needed GI Obstruction- If BILIOUS vomiting- like Malrotation Appendicitis- Vomiting with Appendicitis type pain

Answer 81

If more acute- Diabetic Ketoacidosis If more chronic- Migraine/ Chronic Vomiting Early morning headaches- RAISED ICP In teenagers- consider pregnancy (CHECK PREGNANCY FIRST) , drugs and alcohol

Answer 82

An Upper Respiratory Tract Infection

Answer 83

Duodenum has a blind end and is NOT patent as a result. So it obstructs the Duodenum - Down's Syndrome - Other Intestinal Atresias - VACTERL association (Vertebral defects, Anal atresias, Cardiac defects, Tracheooesophageal fistula, Renal anomalies and Limb abnormalities)

Answer 84

May be associated with Polyhydramnios as the baby can not ingest amniotic fluid normally Distended Abdomen Vomiting may be Bilious or Non-bilious

Answer 85

Abdominal X ray (Double Bubble) - One gas bubble visible in the Stomach, and one in the proximal part of the Duodenum, proximal to the Atresia - Due to air from the stomach being between the Pyloric sphincter and the blind end of the Duodenum Management- Surgical Repair- Duodenoduodenostomy (reconnecting proximal and distal segments of duodenum)

Answer 86

The reversal of a left-to-right shunt to a right-to-left shunt So it is only seen in patients with a shunt to begin with-, like AtrioSeptal Defect So it is an Acquired Right-to-Left Shunt This is due to Increased Pulmonary Pressures changing the Pulmonary Vasculature and leading to Pulmonary Hypertension

Answer 87

Occurs in LATE TEENS Includes Cyanosis and Right Heart Failure Management- Best treatment by prevention by promptly treating Left-to-Right Shunts - Otherwise treat the Syndrome itself with Heart-Lung Transplant or Palliation

Answer 88

Holosystolic Murmur loudest at Left Sternal Edge

Answer 89

Hand, Foot and Mouth Disease- Coxsackie A Myocarditis- Coxsackie B Poliomyelitis- Poliovirus - Mostly Asymptomatic - A quarter of children have Upper Respiratory Tract symptoms - MAY be PARALYSIS if the CNS is affected Meningitis- Echovirus can cause an Aspectic Meningitis

Answer 90

Weight- - 1-5 years old= (Agex2)+8 - 6-12 years old= (Agex3)+7 Energy- 4J/kg Tube size- (Age/4) +4 (cm) Fluids- 20ml/kg on normal Saline bolus (10ml/kg if Trauma or Heart Failure) Lorazepam- 0.1mg/kg Adrenaline- 10micrograms/kg Glucose- 2ml/kg of 10% dextrose

Answer 91

It is the second most common bone cancer in children It starts with LOWER body bones- - Bone pain at NIGHT - Mass or swelling - Restricted movement in a joint

Answer 92

Bone Biopsy is Definitive - Histology will show small blue round cells with clear cytoplasms Xray shows LAMELLATED PERIOSTEAL REACTION (onion skinning) MRI shows a large mass with Necrosis Management- Surgery, Radiotherapy and Chemotherapy

Answer 93

Meningitis Traumatic Brain Injury Haemorrhage

Answer 94

Still's Murmur Venous Hum Acute Infective Illness The 6 S's - Soft - Systolic (as ALL diastolic murmurs are pathological) - Sensitive (changes with child's position and respiration) - Short (not holosystolic) - Single (no additional sounds) - Small (localised, non radiating)

Answer 95

Often made prominent when the child has a Febrile Illness Management- the best option is to review the child in a few weeks. If the murmur persists, then Echocardiogram.

Answer 96

Febrile seizures are seizures associated with fevers They are usually SHORT and Tonic Clonic Management- - Manage seizure like all other seizures- Benzodiazepam - Find the Source of the fever and treat that - Parents should be advised on Anti-Pyretics and not to use them prophylactically Prognosis- it does NOT increase the chances of Epilepsy if simple. Complex= lasts for a LONG time or happens MULTIPLE times with the same fever 30-40% recurrence chance Caused by Roseola Infantum

Answer 97

1st 10 kg of bodyweight- 100ml/kg/day 2nd 10kg of bodyweight- 50ml/kg/day Remaining bodyweight- 20ml/kg/day Sodium- 2-4mmol/kg/day Potassium- 1-2mmol/kg/day Acute Fluid Resuscitation- 10ml/kg (give 2 then call for help)

Answer 98

Percentage dehydration- ((Normal Weight- Current Weight)/Normal Weight) multiplied by 100 Multiply % dehydration by their normal weight (just the number, don't divide by 100) (fluid deficit) then multiply this by 10 (So basically Normal Weight-Current Weight x 1,000) Give this fluid over 1-2 days with their normal fluids

Answer 99

It is a condition resulting from In-Utero Alcohol Exposure Signs- - Functional/ Structural Nervous System Abnormalities (decreased cranial size, structural brain abnormalities, problems with attention, cognition) - Growth Impairments (low birth weight, decelerating birth weight over time) - Specific facial abnormalities (short palpebral fissures, smooth philtrum and thin upper lip) There is NO CURE- just to support throughout life

Answer 100

Caused by CGG repeat in the FMR1 gene on the X chromosome Signs- (Socially Awkward Clicking Horse) - Long face and Large protruding ears - Intellectual impairment - Macroorchidism (Large testes) - Social Anxiety and Autism Spectrum - MITRAL VALVE PROLAPSE - Echolalia (Repeating what you say) Management - Support throughout life- MDT

Answer 101

1) They are mature and intelligent 2) They can not be persuaded to tell the parents or allow the doctor to tell them 3) They are likely to have sexual intercourse with or without contraception 4) Their mental health will suffer unless they receive advice 5) The advice or treatment is in their best interests

Answer 102

Signs- - Milky vomit after feeding - Crying/ Irritability - Arching of the back and Drawing up of the KNEES TO CHEST (These movements may be mistaken for Seizures) Management- - Keep baby upright after feeding and keep cot on an incline Medical- GAVISCON and OMEPRAZOLE (Quesmed also said Alginate) Surgical- *FUNDOPLICATION*

Answer 103

Common Cause of DIARRHOEA and VOMITING after ingestion Bacterial- - Staphylococcus Aureus- cooked meats and creams - Cereus- Reheated Rice - Perfringens- Reheated or cooked meat - E-coli - if recently TRAVELLED to ASIA/ AFRICA Viral- - Rotavirus (most common cause in infants) - Norovirus (most common cause in all ages) - Adenoviruses (resp infections as well)

Answer 104

Fluid Replacement (WATER) and antibiotics if severe Antibiotics if: - Systemically unwell - Immunosuppressed - Elderly Specific antibiotics (Stay Clean, Cook Everything, Clean Thoroughly) - Salmonella or Shigella- Ciprofloxacin - Campylobacter- Erythromycin - Cholera- Tetracycline

Answer 105

Small vessel vasculitis- in 3-5 year olds Signs- PANAF - Purpura or petechiae on Buttocks and Lower Limbs - Abdominal Pain - Arthralgia (Knee and Ankles usually) - Nephritis (Haematuria and Proteinuria) - Usually happens after an UPPER RESPIRATORY INFECTION - May have fever Diagnosis- Biopsy with immunofluorescence for C3 and IgA- but rarely don- Pick DIPSTICK over this for investigations Management- - NSAIDs for analgesia - Antihypertensives for blood pressure - Urine dipstick for a year to check for renal function

Answer 106

Signs - Alcohol-Induced PAIN in Lymph Nodes - Cervical or Supraclavicular Non-tender Lymphadenopathy - Symptoms caused by compression of surrounding structures- !!!!!!!SHORTNESS OF BREATH/ ABDOMINAL PAIN - B symptoms (weight loss, night sweat, fatigue)- only seen in 30% of patients - Hepatosplenomegaly Diagnosis- - Lymph Node Biopsy- REED STERNBERG CELLS - !!!!!!!!!Low Hb (Anaemia) and Raised LDH is poor prognosis Management- Chemotherapy

Answer 107

Causes- Processus Vaginalis does not close (the path the testes take to descend) Management- Most resolve within a year, so wait ONE YEAR, then surgical correction if still not resolved Prognosis if untreated- Inguinal Hernia

Answer 108

Non-communicating- there is an OBSTRUCTION in the Ventricular system - Congenital Malformation (AQUEDUCT STENOSIS or CHIARI MALFORMATION) - Tumour or Vascular Malformation in the Posterior Fossa - Intraventricular Haemorrhage (consider in PREMATURE infants) Communicating- Failure to reabsorb CSF - Meningitis - Subarachnoid Haemorrhage - Traumatic Brain Injury

Answer 109

Diagnosis- Cranial Ultrasound or CT/MRI of the brain Management- Ventriculoperitoneal Shunt to move CSF to the abdominal cavity (look out in case the shunt gets infected though)

Answer 110

RAISED ICP Signs- - Vomiting - Irritability - Lethargy - Sunsetting of Eyes Hydrocephalus- Tense Anterior Fontanelle, Distended Scalp Veins

Answer 111

Weight Gain Lethargy Erectile Dysfunction and Loss of Libido Depression Gynaecomastia - Associated with Infertility and OSTEOPOROSIS

Answer 112

HRT (give them testosterone) Monitor for- - Polycythaemia (testosterone affects erythrocytosis) - Bone mineral density (DEXA) - Prostate status - LFTs (hormones affect Liver Function)

Answer 113

It is Brain Damage resulting from antenatal or perinatal Hypoxia Primary Neuronal Death (Immediate) and Secondary Reperfusion Injury (Delayed) Caused by anything that causes HYPOXIA - Pre-Partum (PLACENTAL ABRUPTION) - During Delivery (Prolonged Delivery, Cord Compression) - Post-Partum (Prolonged Respiratory Arrest)

Answer 114

Ranging from Mild (Irritability) to Severe (Hypotonia, Poor Responses, Prolonged Seizures) Diagnosis- EEG monitoring and multiple MRI scans Management- - Respiratory Support - Anticonvulsant Therapy - Careful Fluid Balance and Electrolyte Monitoring - The use of INOTROPES - COOLING THE BABY TO INDUCE HYPOTHERMIA protects against Secondary Reperfusion Injury

Answer 115

Autoimmune disease of an unknown cause where the Number of Platelets is reduced In Children it is SELF LIMITING Adults- CHRONIC RELAPSING

Answer 116

Excessive Bruising and Bleeding (NON-Blanching, PETECHIAL RASH) Tiny Reddish-purple Dots May develop this after a Viral Infection Managed with- Platelet Transfusion avoid unless Life-threatening. Steroids are used if Persistent and Splenectomy if Refractory

Answer 117

RECURRENT infections Anaemia (Pallor and Fatigue) Thrombocytopaenia (PURPURIC Rash)

Answer 118

Skin Infection caused by Staph/ Strep Signs- - Pruritic Rashes with GOLD CRUSTING - May also be fever - In Children Management- - Fusidic Acid and Oral Flucoxacillin - It is HIGHLY INFECTIOUS so isolate until 48 hours of antibiotic treatment

Answer 119

Investigations are UNNECESSARY if Asymptomatic Child with Low-Risk Ingestion Otherwise- ENDOSCOPY or OPEN SURGERY

Answer 120

Sudden Onset Shortness of Breath with a Focal Site of the Lungs Especially MONOPHONIC WHEEZE in RIGHT LOWER LOBE

Answer 121

Invagination of small intestine into large intestine Signs- - SUDDEN, SEVERE COLICKY pain and draws legs up - Child recovers but becomes Lethargic Other symptoms- - May refuse feeds - Abdominal Distension - Sausage Shaped Mass in Abdomen - REDCURRENT Jelly Stool

Answer 122

Abdominal Ultrasound- TARGET Sign (Concentric Echogenic and Hyperechogenic Bands) Management- - Rectal Air Insufflation (Air Enema) or Contrast Enema (only to be performed if the child is stable) - Laparoscopic Correction if haemodynamic unstable, PERITONITIS and PERFORATION

Answer 123

Loss of Consciousness >5 minutes Vomiting more than twice and Drowsiness- CT within 8 hours

Answer 124

It is a disease which causes Muscle Weakness and Skin Rash (on areas exposed to sunlight- face, back, limbs) Signs- 1) Fatigue 2) Joint Pain 3) Weakness of Proximal Muscles 4) Malar Rash 5) Heliotrope Rash over eyes (Purple Rash over Eyes)

Answer 125

Investigations - High ESR and Creatine Kinase - POSITIVE ANAs Diagnosis can be confirmed via -MRI (Muscle Oedema) -Muscle Biopsies -Nailfold Capillaroscopy showing Microangiopathy Management- - Steroids for flares - Steroid-sparing immunosuppression for maintenance (Methotrexates or Biologics)

Answer 126

It is the MOST COMMON CAUSE of CHRONIC JOINT PAIN in Children Signs- - Pain with (3) (Fevers, Malaise, Salmon pink Rash) and Joint Pain

Answer 127

Management- - MDT input needed - Walking Aids needed, may need Psychological support to help with coping as well - Medical management- 1) NSAIDs for Symptoms 2) Oral or IV Corticosteroids (Prednisolone) if Systemic Symptoms, if NO Systemic Symptoms (Intra-articular Methylprednisolone Injection) 3) Steroid-sparing agents (Methotrexate, Biologics) Complications- 1) Flexion Contractures 2) Joint Destruction 3) Growth Failure (from the STEROIDS and CHRONIC DISEASE) 4) Anterior UVEITIS

Answer 128

It is an AUTOIMMUNE MEDIUM-VESSEL VASCULITIS Seen in UNDER 5 YEAR OLDS USUALLY Signs- CRASH and Burn - Conjunctivitis - Rashes - Adenopathy (Lymphadenopathy) - Strawberry Tongue (and Cracked Lips) - Hands and Feet Red, Swollen and Skin Peeling and High Fever Usually very unwell

Answer 129

Intravenous Immunoglobulin and Aspirin (high dose) Surveillance of Coronary Artery Aneurysm (ECHOCARDIOGRAM for THIS)

Answer 130

Intravenous Immunoglobulin and Aspirin (high dose) Surveillance of Coronary Artery Aneurysm (ECHOCARDIOGRAM for THIS)

Answer 131

SAM TAM QQ - Sotalol - Antipsychotics - Metoclopramide - Tricyclics - Amiodarone - Macrolides (-Mycins) - Quinolones (-Floxacins) - Quinidine

Answer 132

10 small squares Cardiologist should supervise them to see whether they need an Implantable Defibrillator BETA BLOCKERS may help

Answer 133

The Midgut rotates and fixes in an abnormal position- this makes it more likely for VOLVULUS to occur and the duodenum is likely to be compressed by LADD's BANDS SIGNS- - Bilious Vomiting within the FIRST DAY of LIFE (as the gut is already developed like that from the start) - Distension and Constipation

Answer 134

Diagnosis- Upper GI Contrast Study shows Corkscrew in proximal bowel. Contrast can not pass beyond this area Management- Urgent Surgery

Answer 135

It is the most common Congenital GI Abnormality- It is a remnant of the Vitello-Intestinal Duct of the Embryo (which should completely disappear at 6 weeks gestation) It is a True Diverticulum (contains all 3 layers of the bowel wall) It presents in INFANTS, not in NEONATES

Answer 136

Symptomless (found in operation) May be BILIOUS (GREEN) Vomiting If Patent Vitello-intestinal Duct- may be discharging intestinal content PAINLESS Rectal Bleeding (most common cause in Children)- due to potential ectopic pancreatic tissue that may also form in there RIF Abdominal Pain (due to diverticulum inflammation) THREE COMPLICATIONS- Intussusception (may act as the APEX for ILEOILEAL TYPE) Obstruction- if it becomes trapped in a Hernia Perforation by foreign body

Answer 137

99mm Technetium scan CT scan Small Bowel Enema May show FLUID FILLED POUCH on ULTRASOUND Management - Treat Clinical Presentation (Obstruction with NG Tube) - Resection of Diverticulum (Wedge Excision or Small Bowel Resection and Anastamosis)

Answer 138

Conscious and Coughing- - Encourage them to cough Conscious but can't Cough- - 5 back blows and 5 chest (infant), abdominal (child) thrusts Unconscious- - 5 rescue breaths and CPR

Answer 139

Croup- - Cough and Fever and inspiratory stridor in Moderate and Severe Cases - Symptoms WORSEN at NIGHT, which is when patients typically present Epiglottitis- - Fever, inspiratory stridor and Drooling and Struggling to breath - Normally VERY IRRITABLE- it is not advised to disturb these children as they will cry Bacterial Tracheitis- - Symptoms between Croup and Epiglottitis - Difficulty Breathing and Fever

Answer 140

Maternal Rubella (and other infections) Drugs (Thalidomide, Isotretinoin, Lithium) and Alcohol Maternal Diabetes (poorly controlled Type 1 or Type 2 Diabetes- NOT cos of Gestational Diabetes)

Answer 141

A Large Right Atrium, a Small Right Ventricle due to Low Insertion of the Tricuspid Valve

Answer 142

Peripheral and Central Cyanosis Respiratory Distress !!!!!!!!!!!!!!!!!!A single loud S2 (+maybe patent ductus arteriosus murmur) Chest X ray shows Egg-on-a-String appearance ("bulbous" upper portion that represents the enlarged pulmonary artery, and a "pinched" lower portion that represents the narrowed aorta. This appearance is caused by the anterior position of the pulmonary artery and posterior position of the aorta, and can be a helpful diagnostic clue for TGA.)

Answer 143

Fever> 40 degrees Coryzal (cold-like symptoms) Conjunctivitis then Maculopapular rash (Face and Trunk) 2-5 days after symptoms KOPLIK SPOTS- small grey spots in mouth

Answer 144

1) Measles specific IgM and IgG serology (ELISA) is most sensitive after 3 days 2) Measles RNA detection by PCR (best for 13 days after rash)

Answer 145

Management- - Antipyrexial medication - Vitamin A (if under 2 years old)- think Vit A helps the eyes and Measles affects the eyes first (Conjunctivitis) - Ribavarin (but not routinely used) Complications- (Ear, Lung, Head) - Acute Otitis Media - Bronchospasms - Encephalitis

Answer 146

It is a condition where the first poop is so thick that it does not pass within the first 2 days BILE-STAINED VOMIT and DISTENDED ABDOMEN It is associated with Cystic Fibrosis In Abdominal Xray- you will see a BUBBLY APPEARANCE of the INTESTINE with a lack of air-fluid levels Management- - Drip and Suck (IV Fluid and Stomach Drainage with Ryles Tube) with enemas to remove the Meconium - Surgery needed in Severe Cases

Answer 147

Streptococcus pneumoniae (Positive) Neisseria meningitidis (Negative) Haemophilus influenzae Listeria monocytogenes (extremities of age)

Answer 148

Echoviruses Coxsackie A and B Polio

Answer 149

Fungal- Cryptococcus and Mycobacterial Parasitic- Acanthamoeba (associated with Keratitis) and Toxoplasma Non infective- - Malignancy - Chemical Meningitis - Drugs (NSAIDs and Trimethoprim) - Sarcoidosis - Systemic Lupus Erythematosus - Behcet's Disease (inflammation of blood vessels- consists of ulcers, itchy eyes and acne)

Answer 150

Headache Fever Neck Stiffness Photophobia Nausea and Vomiting FOCAL NEUROLOGICAL SIGNS SEIZURES

Answer 151

Kernig's and Brudzinski's These tests are not sensitive so sometimes patients may not have these signs and still have meningitis

Answer 152

Bacterial- 2g IV Ceftriaxone (twice daily), add IV Amoxicillin if very young or very old to cover Listeria. If <1 month, Cefotaxime can be used Viral- IV Acyclovir Fungal- Amphotericin, Flucytosine and Fluconazole If community-based, IM/IV Benzylpenicillin may be used if there is a Non-blanching Rash while waiting for Hospital transfer. so give this FIRST in GP If Penicillin-allergic, !!!!!!Chloramphenicol may be used

Answer 153

Blood cultures, gas and test Arterial blood gas CT HEAD and LUMBAR PUNCTURE Blood tests

Answer 154

Bacterial - Clear or Turbid - HIGH OPENING PRESSURE - usually Neutrophil Leukocytosis - 100-200 PMN (Polymorphonuclear leukocyte) - Positive culture results (may be negative) - High Protein (due to bacterial contamination) - Low Glucose (<0.4g/L) (bacteria use it as an energy source) (LESS THAN HALF of SERUM GLUCOSE) Aseptic (Viral) Meningitis - Clear or Slightly turbid with 15-100 Leukocytes - usually Lymphocytic Leukocytosis - Negative culture results (cos Aseptic) - 0.5-1g/L (<100mg/L) of proteins with Normal Glucose (>0.4g/L) (as Viruses use cell machinery to replicate) Tubercular Meningitis (history of recent foreign travel) - Clear or Slightly Turbid - Fibrin web may develop - 30-500 lymphocytes plus PMNs (but apparently it is actually MONONUCLEATED), with NEGATIVE gram stain - High Protein - Low Glucose Cryptococcal Meningitis - HIGH OPENING PRESSURE - May give any of the results above so always think of this as a possible differential if they have HIV or are immunocompromised - Cryptococcal Antigen testing or India Ink Staining should be requested

Answer 155

Disseminated Intravascular Coagulation (look out for Conjunctival Haemorrhages) Subdural Effusions Syndrome of Inappropriate Antidiuretic Hormone Secretion Seizures/ Coma - Hydrocephalus, Hearing loss, Ataxia, Blindness, Cranial Nerve Dysfunction

Answer 156

Viral causes less symptoms than Bacterial

Answer 157

Protein= Up to 1.5g/L Glucose= 2.5-4.4 mmol/l Opening pressure= (10-25 cmH2O) WCC= 0-8

Answer 158

Neisseria- Non blanching rashes Strep Pneumoniae- Ear Infections

Answer 159

Altered Mental State (not seen in Meningitis) Fever Convulsions FOCAL NEUROLOGICAL SIGNS Headache with MENINGISMS

Answer 160

Blood cultures Arterial blood gas CT HEAD and LUMBAR PUNCTURE Blood tests

Answer 161

2g IV Cefriaxone and 10mg/kg IV Acyclovir

Answer 162

Generalised Fatigue/ Malaise (common) GI Disturbances (common) Photosensitivity (so can make this worse in Meningitis) and urticarial rash (common) Renal Failure Hepatitis Haematological abnormalities

Answer 163

Bilateral Multifocal Haemorrhages in the Temporal Lobes

Answer 164

Meningococcal Infection

Answer 165

Meningococcaemia Meningitis (lethargy, headache, fevers, vomiting) Mixture of both- associated with a rapidly developing Purpuric Rash Hypovolaemic Shock (cold peripheries, poor cap refill, tachycardia, low urine output) WATERHOUSE-FRIDERICHSEN (adrenal haemorrhage and septic shock also happen)

Answer 166

Waterhouse-Friderichsen Syndrome

Answer 167

Blood or CSF Cultures PCR testing for Neisseria

Answer 168

Broad Spectrum Antibiotics PICU admission if Septicaemia Ciprofloxacin or Rifampicin for household contacts

Answer 169

Sepsis (tachycardia, hypotension, tachypnoea, petechial rash) (bear this one in mind) Raised ICP (remember the Victor VILS story) Focal Neurological Signs (bear this one in mind) Coagulation Disorder Cardiovascular Instability Respiratory Failure

Answer 170

Intravascular Coagulation

Answer 171

The inflammation of Mesenteric Lymph Nodes Signs- - Diffuse Abdominal Pain following a previous Upper Respiratory Tract Infection (BUT may be RIF pain as well) - Low Grade fever with general abdominal tenderness - Also Diarrhoea, Vomiting, Fatigue and Loss of Appetite - Pharyngitis in the mouth MAY be there - US Abdomen shows ENLARGED MESENTERIC LYMPH NODES - Rule out Appendicitis based on Clinical Presentation (MORE SEVERE and not after URTI) Management- - Monitor them overnight to look for worsening of their clinical status - Mesenteric adenitis will also improve spontaneously, whereas appendicitis will not

Answer 172

It is the body being unable to properly metabolise Galactose, which leads to toxic levels that accumulate in the blood It presents in the first 1-2 weeks of life (DIARRHOEA, VOMITING and LETHARGY) Management- - Remove Galactose and Lactose from their diet otherwise can lead to NEUROLOGICAL complications This is screened for at the Newborn Blood Spot Test

Answer 173

It is an enzyme defect leading to the accumulation of BRANCHED AMINOACIDS This leads to KETOACIDS in the bloodstream which accumulates in the urine giving a MAPLE SYRUP SMELL Symptoms- LETHARGY, POOR FEEDING ABILITY and IRRITABILITY Management- - Tight Lifelong diet to prevent build up of branched aminoacids (valine, leucine and isoleucine) otherwise can lead to NEUROLOGICAL COMPLICATIONS This is screened for at the Newborn Blood Spot Test

Answer 174

It is a disorder affecting Fatty Acid Oxidation where the body's ability to convert Fatty Acids to Acetyl-Coenzyme is affected This is AUTOSOMAL RECESSIVE It presents as (LOW KETONES, LOW GLUCOSE and LETHARGY) Management- - Avoid FASTING and eat SIMPLE CARBS This is screened for at the Newborn Blood Spot Test

Answer 175

Medium chain acyl-coA dehydrogenase deficiency Galactosaemia Maple Syrup Disease Sickle Cell Disease Congenital Hypothyroidism Cystic Fibrosis

Answer 176

It is the MOST COMMON FORM of NEPHROTIC SYNDROME in children (Proteinuria, Oedema, Hypoalbuminaemia) Also Hyperlipidaemia Signs- - OEDEMA (facial swelling usually) - Frothy Urine (cos of the proteinuria) - If a child has nephrotic syndrome, assume it is Minimal Change Disease Investigations- Urine Dipstick for Proteinuria/ Haematuria Management- - Corticosteroid (Prednisolone) - then other immunosuppressive agents (Ciclosporin) - FLUID RESTRICTION and reduced SALT INTAKE - Human Albumin and Furosemide

Answer 177

Spontaneous Peritonitis Thrombosis (high proteins) Renal Damage (cos it is a kidney disease) Increased Risk of Infections

Answer 178

Minimal or No changes to Histology on biopsy

Answer 179

A range of conditions where the muscles break down and get weaker over time The most common form of this is Duchenne's Muscular Dystrophy Causes- - Mutations that reduce Dystrophin expression (protein involved with contraction) - Duchenne's- protein is NOT express, Becker's- expressed at LOW LEVELS They are X LINKED RECESSIVE

Answer 180

Duchenne's - Muscle Wasting (and weakness) in early childhood, also the GAIT is OFF - Usually wheelchair bound before puberty and they DIE from RESPIRATORY FAILURE by early 20s - They may appear BULKY- as fat REPLACES muscle in these cases - The child may SLIP THROUGH parents' hands when they pick them up (due to loose shoulder muscles) and they WALK THEIR ARMS when getting up Becker's - Muscle wasting and weakness presents in LATE childhood- they usually survive oto their 30s

Answer 181

First Line- Creatine Kinase Genetic Testing detects Duchenne's and is the gold standard Muscle biopsies are sometimes performed but Genetic testing is better Management- just support and therapy

Answer 182

The bowels of premature infants becomes Ischaemic and Infected

Answer 183

Prematurity and Low Birth Weight Non-breast milk feeding Sepsis Hypoxia and Poor Intestinal Perfusion

Answer 184

Presents in PREMATURE infants in the first 3 weeks of life Bile-streaked vomiting and Bloody diarrhoea Abdominal Distension with Absent bowel sounds (cos there is air) Systemic Compromise (like ACIDOSIS) Diagnosis- - Abdominal Xray (Dilated bowel loops and Pneumatosis intestinalis (gas in intestine which dilates it), Portal Venous Gas and Pneumoperitoneum (so gas everywhere basically)) - Staged using Bell's classification Management- (Antibiotics and Airways, Control their feeding and arrange for surgery) - Nil by mouth and give them a Nasogastric Tube - Broad-spectrum antibiotics - Give them Total Parenteral Nutrition to rest the bowel - Supportive Treatment with IV Fluids and Ventilation is crucial - Surgery to Resect Necrotic Sections of the bowel may be necessary- especially if there are PERFORATIONS. Contact the surgeons early How to prevent this- - BREASTFEED and DELAY UMBILICAL CORD CLAMPING

Answer 185

24 hours- 14 days- Physiological Jaundice (high UNCONJUGATED bilirubin) Breast Milk Jaundice (no symptoms- just Jaundice) Infection Dehydration Polycythaemia, Bruising or Haemolysis Crigler-Najjar Syndrome (liver can't break down bilirubin so it builds up) >14 days- Physiological Jaundice Breast Milk Jaundice (no symptoms- just Jaundice) Infection HYPOTHYROIDISM Biliary Obstruction (Biliary Atresia) Neonatal Hepatitis

Answer 186

Elevated Bilirubin due to - Babies may just be Polycythaemic at birth - Red cell life span of newborns is shorter than adults - Hepatic Bilirubin metabolism is less efficient for the first few days

Answer 187

Measure their bilirubin levels 1) Depending on this- Phototherapy may be done (repeat bilirubin in 24 hours if this is the case). After starting Phototherapy, monitor bilirubin levels regularly in 4-6 hours - once they are >50 micromoles below the threshold, then stop phototherapy and check again- if it still below >50 micromoles below the threshold then no further action needed. 2) If significantly high, then Exchange Transfusion should be done If not managed, it can lead to KERNICTERUS

Answer 188

A build up of Unconjugated Bilirubin

Answer 189

Direct Coomb's Test to look for Haemolytic Disease of the Newborn (Rhesus Negative and ABO Incompatibility)

Answer 190

Breastfeeding

Answer 191

It is caused by a lack of Alveolar Surfactant This is produced from 26 weeks gestation so PREMATURE babies are at high risk of this Diagnosis- - Generally based on history- Neonatal Respiratory Distress Syndrome (Grunting, Flaring, Intercostal Recession) - CXR- ground glass Management- - Intratracheal Instillation of ARTIFICIAL SURFACTANT - Give the mother Glucocorticoids (Corticosteroids) if preterm delivery is suspected

Answer 192

Stillbirth- Death after 24 weeks of gestation- before or during birth Neonatal Death- Death within first 28 days of life Perinatal Death- Stillbirth plus Neonatal Death

Answer 193

Stillbirth- Foetal Growth Restriction Neonatal Death- Preterm Birth

Answer 194

1) Birth 2) Dry the baby and perform the Initial Check (tone, breathing, heart rate) 3) If not breathing, try PHYSICAL STIMULATION FIRST, then open airway and give 5 artificial inflation breaths and consider Oxygen (but generally avoided in newborns) 4) See if this helps 5) If chest is not moving, optimise the airway control by repeating INFLATION < 60- Ventilate, Compression, IV drugs 6) When the chest is moving, ventilate for 30 seconds if HR<60 7) If HR is still< 60 start chest compressions (3:1 rate) 8) If HR still<60 start IV drugs

Answer 195

Early Onset- - Group B Strep if Ascending microorganisms from Cervix - Colonises Urinary Tract - Can cause Asymptomatic Bacteriuria or UTI in the mother - Treat with IV BENZYLPENICILLIN Intrapartum if 1) Previous baby had GBS, Maternal Fever, Positive swab in pregnancy Transplacental- CMV, Rubella, Listeria, Toxoplasma Late onset- - Staph Aureus commonly

Answer 196

Prematurity Rupture of membranes> 18 hours for pre-term babies Rupture of membranes> 24 hours for term babies Maternal Intrapartum Fever Chorioamnionitis

Answer 197

BLOOD CULTURE before antibiotics- also FBC and CRP - BENZYLPENICILLIN and GENTAMYCIN Strongly consider LUMBAR PUNCTURE - Chest Xrays only if there is strong suspicion of a Resp source - URINE CULTURE if late onset (>72 hours) Repeat CRP 18-24 hours hours after initial Antibiotics

Answer 198

Diabetes Mellitus Urinary Tract Infection Constipation (due to compression of the bladder)

Answer 199

History, Examination and Urinary Dip should always be performed If secondary- Urine dip, Urine Osmolarity and Renal Ultrasound Management - Reassure that this is normal - Nocturesis Alarm - If over 7 and alarm doesn't work, a drug called Desmopressin- DDAVP (a type of synthetic ADH) can be given

Answer 200

It is any bodily injury in a child that had been deliberately afflicted on them or any injury where the caregiver has failed to prevent such an injury Investigations- Radiology and Bloods (exclude organic causes such as clotting disorders or haematological malignancy) Management- Inform a senior and admit the child for safeguarding

Answer 201

Oesophageal Atresia- blind-ended oesophagus Trachek-oesophageal Fistula- there is communication between the oesophagus and the trachea Most patients that present with them have both

Answer 202

Antenatal- POLYHYDRAMNIOS as fluid cannot pass to the baby to be absorbed Postnatal- - Respiratory Distress - Distended Abdomen - Choking/ Problems with swallowing - Difficulty with passing NG tube down Check with NG Tube and Chest Xray Management- Surgery

Answer 203

Child shows persistent defiant and hostile behaviour towards FIGURES of AUTHORITY like a parent or teacher Managed with Therapy

Answer 204

It is the most common bone cancer in children Presentation- - Pain and Swelling with Prolonged Onset that occurs in the Metaphyses of Long Bones (The ends of Long Bones). Pain is worse at morning and night Diagnosis- - First- Urgent Xray shows new bony growth and periosteal reaction which causes a Sunburnt Appearance - Definitive diagnosis- Bone Biopsy Management- surgery, radiotherapy and chemotherapy

Answer 205

Call for help Open airways If breathing but not normal- 5 rescue breaths If no signs of life- 15 chest compressions and 2 rescue breaths Repeat 15:2

Answer 206

Airway Positions- - Infants- Neutral position - Young children- "sniffing the morning air" - Older children- Head tilt chin lift and Jaw thrust Differences- - Paediatrics first intervention is 5 rescue breaths - Children are more likely to suffer from RESPIRATORY ARRESTS rather than CARDIAC ARRESTS

Answer 207

Pulses- - Carotid Pulses in >1 year olds - Brachial or Femoral pulses in Infants How to perform Chest Compressions- - 100-120 compressions per minute - Make sure there is full recoil of the chest - Infants- 2 fingers from one hand or both thumbs - Small children- One handed compressions with the Heel of your hand - Larger children- Two handed compressions with Interlocking hands- like an Adult Compression depth- - Babies and young children- 1/3 of chest depth - Adult children- 5cm

Answer 208

Infants- - Difficulty feeding and Faltering Growth - Poor Appetite - Abdominal Pain and Vomiting - Fatigue - Hepatomegaly (more evident than oedemas and cyanosis) Children- - Exercise Intolerance - Fatigue - Hepatomegaly

Answer 209

Investigations- - Bloods (BNP, Magnesium, Bone Profile, TFTs, CRPs, LFTs, FBC, U&Es) - Imaging- Chest Xray and Echocardiogram - ECG Management- (reduce fluid in, increase fluid out AND surgical) - Fluid Restriction and Feeding Plan from Dietician - Diuretics with Inotropic Support if Needed - Surgical- Correction of Defect and Heart Transplant in End-stage cases

Answer 210

Trisomy 21- SEPTAL DEFECTSs- mainly ATRIOVENTRICULAR SEPTAL DEFECTS Foetal Alcohol Syndrome- Ventricular Septal Defects Edward's Syndrome (Trisomy 18) - (Ventriculo) Septal Defects DiGeorge Syndrome (22 q Deletion)- Aortic Arch Defects Turner Syndrome (45XO)- Bicuspid Aortic Valves and Coarctation of the Aorta Noonan Syndrome- Pulmonary Stenosis Patau Syndrome- Dextrocardia (heart is on the right instead of the left) (also PDA, VSD, ASD etc)

Answer 211

Biological mother usually If someone does not have Parenteral Right, you either start a new agreement or speak to someone who used to have Parental Right Father has Parental Rights if (3) - Married to the child's mother at the time of birth - Listed on the birth Certificate - Is in a Parental Responsibility agreement Adoption- both parents have PR if they have both jointly adopted Same-sex partners- - If civil partners at time of fertility treatment- both have PR - If not civil partners, the second parent can obtain PR by 1) applying for Parental Responsibility 2) becoming Civil Partners and jointly registering the birth 3) becoming Civil Partners and forming a Parental Responsibility Married Step parents or Registered Civil Partners- - Can obtain PR via Parental Agreement or spplying through courts

Answer 212

If awarded by courts, only the courts can remove them Parental Responsibility is NOT LOST through divorce, loss of contact with the child or lack of financial contribution

Answer 213

Fifth Disease Slapped Cheek Syndrome Erythema Infectiosum Signs- - Prodrome of FEVER, CORYZA and DIARRHOEA - Arthralgia - a LACE-LIKE RASH develops across the body with RED RASH on CHEEKS (may not be present in adults and would only present with Arthralgia) Management is Supportive Complications 1) Red cell aplasia (they didnt develop properly) - Parvovirus infection reduced Erythropoeisis and is NOT significant for most patients, however in VULNERABLE groups that rely on Erythropoeisis (like Sickle Cell Anaemia and Hereditary Spherocytosis) it can cause SEVERE ANAEMIA which can cause an APLASTIC CRISIS 2) Infection in first half of pregnancy can cause Severe Foetal Anaemia which precipitates Hydrops Foetalis and Miscarriage 3) Cardiomyopathy

Answer 214

The ductus arteriosus is open, even after 4 weeks of life - May be asymptomatic or present with signs of HEART FAILURE - Breathing issues and bradycardia is COMMON - NO CYANOSIS- remember it is one of the Acyanotic Conditions - Collapsing Pulse - Unlikley to have Radio-femoral delay or Clubbing - A MURMUR is heard- continuous MACHINE WHIRRING MURMUR throughout the cardiac cycle Management- - Only needed if the baby is Symptomatic - 1/3 of patients need an NSAID- Paracetamol may be used instead as well - Medical Closure is more successful in Premature babies - 8% will need Surgical Ligation

Answer 215

It is the AVASCULAR NECROSIS of the femoral head occurring in children aged 4-8 years old It presents with GRADUAL ONSET LIMP and HIP PAIN. The pain can also be referred to the knee. Transient Synovitis pain would be similar but would only last up to 2 WEEKS. If >4 WEEKS, suspect PERTHE's Worsening Hip pain with no other symptoms- pain hurts on passive movement

Answer 216

Diagnosis- Frog Leap Hip Xray shows INCREASED DENSITY of the FEMORAL HEAD, SCLEROSIS and FRAGMENTATION of the EPIPHYSIS. An Xray may be normal however Management- If <50% of the femoral head is involved- Bed Rest and Traction. >50% of the femur head is involved, PLASTER CAST to keep the hip abducted or OSTEOTOMY

Answer 217

It is a severe URTI characterised by bouts of SPASMODIC COUGHING which can lead to apnoea in infants, followed by gasping for breath Caused by Bordetella Pertussis It presents as a cough- where there is a PROLONGED PERIOD OF COUGHING PER EPISODE There will also be - Inspiratory Whooping (coughing for ages, followed by massive gasp) - Rhinorrhoea - Post-tussive Vomiting (Coughing after vomiting) - Apnoea Also High RR and Fever as expected

Answer 218

Prevented by the Pertussis Vaccination- although there may be breakthrough cases despite vaccination Diagnosed via PCR Management- - First line= Macrolides, unless >21 days then Paracetamol - If antibiotics are started late, they may not alter the course of illness but may minimise transmission to other patients

Answer 219

It is an AUTOSOMAL RECESSIVE MUTATION in the Phenylalanine Hydroxylase enzyme. This causes a high amount of Phenylalanine to build up to toxic levels in the blood which DAMAGES the NERVOUS SYSTEM Signs- - Seizures - Poor Growth - Hypopigmentation- leading to Fair Skin and Hair - Musty Body Odour Long term complications- - Intellectual impairment - Hyperactivity - High risk of Eczema

Answer 220

It is screened for on the Blood Spot Test within the first week of life Management- - Lifelong Low Protein Diet and Supplemental amino acids to avoid Phenylalanine - They must also avoid the artificial sweetener Aspartame as this becomes converted to Phenylalanine (this is found in diet fizzy drinks, sugar-free squash drinks and chewing gums)

Answer 221

It is a condition where the infant is born with Micrognathia (small jaw), Glossoptysis (Posterior Tongue) and a Cleft Palate. Therefore, the infants may have breathing or feeding difficulties after birth Management- - Airway or feeding support. Physiological effects usually resolve within 3-6 months but surgical repair may be needed- specifically for the cleft palate. Most babies will go on to lead a normal life

Answer 222

Renal- - Flank pain and Haematuria= Cyst Rupture - Flank pain, Fever and Urinary Symptoms= Cyst Infection - Hypertension - Renal Failure (Slowly progresses to CKD) Extra-renal- - Extrarenal cysts- Liver, Pancreas, Spleen - Intracranial Berry Aneurysms- commonly at the junction of the Anterior Communicating Artery and Anterior Cerebral Artery - Mitral Valve Prolapse, Aortic Regurgitation - Diverticular Disease

Answer 223

Cyst Rupture Cyst Infection CKD Subarachnoid Haemorrhage (due to aneurysm) Intracerebral Haemorrhage (due to hypertension)

Answer 224

Investigations- - Ultrasound (40, 60, 60+)- 324 1) 15-39 years old> 3 cysts 2) 40-59 years old >2 cysts bilaterally 3) >60 years old> 4 cysts bilaterally - CT/MRI can determine the extent of the disease Management- - Supportive management of CKD - Treat the hypertension - Tolvaptan slows the Formation of Cysts

Answer 225

Caused by Pressure in utero due to Oligohydramnios When Oligohydramnios occurs due to Bilateral Renal Agenesis, it is called Potter's Syndrome (SQUASHED FACE because of low amniotic fluid) Flattened "parrot-beaked" nose Recessed chin Downward Epicanthal Folds Low-set, Cartilage deficient ears Pulmonary Hypoplasia

Answer 226

It is a condition caused by Genetic Imprinting (meaning genes from a specific parent are interpreted differently) A gene on Chromosome 15 is imprinted in a way that the child only expresses the maternal gene (Either deletion of the paternal copy or Disomy of the maternal copy) ("Prada has no Father") If the same thing happens with the maternal copy then it is called Angelman

Answer 227

H - Hypotonia U - Undescended Testicles and Hypogonadism S - Short Stature and Poor growth/ feeding initially L - Learning Disability H - Hyperphagia and Obesity in older child Diagnosis- genetic testing Management- MDT approach (locking up fridges and giving Growth Hormone for Short stature)

Answer 228

It is when puberty occurs before 8 years in girls and 9 years on boys Gonadotropin-Dependent Precocious Puberty (GDPP)- - Idiopatic (90% of cases) - Brain Tumours and Cranial Radiotherapy - Any form of Structural Damage- Hydrocephalus, Post Infection (Meningitis), Traumatic Head Injury Gonadotropin-Independent Precocious Puberty (GIPP)- - Gonadal Tumours - Adrenal or Liver Tumours - Congenital Adrenal Hyperplasia

Answer 229

Investigations- (done by specialist) - Testosterone - Adrenal Androgens - Brain MRI - Pelvic Ultrasound - Intra-abdominal imaging if Adrenal or Hepatic tumour is suspected - Bone Age Management- - GnRH analogues (-relin) If left untreated- Reduced Final Height and Psychology of child is also affected

Answer 230

Boys- Enlargement of Testes >4ml Girls- Breast development

Answer 231

Being Immunocompromised (Diabetes) and Having other Skin Conditions It is a Bacterial infection of the Dermis and Subcutaneous Tissue of the EYE AREA caused by Strep or Staph

Answer 232

- Erythema, OEDEMA, Calor, Pain over the EYES - Poorly Demarcated Margin - Systemic Upset (Fever, Malaise) - Lymphadenopathy - some kind of Skin Breach (Ulcer, Wound) Orbital Cellulitis- Visual Symptoms and Eye Movements are limited and there is ALSO a HEADACHE - there will also be Erythema and Oedema. If No Visual Symptoms= Preseptal

Answer 233

CT SCAN to check the depth of the infection and whether the CNS is affected- then IV Co-amoxiclav Blood Tests and Cultures (NOT CSF) Skin Swab for Culture Oral or IV Co-Amoxiclav (Do these asap) Mark the area of Erythema to aid in the detection of rapidly developing cellulitis Elevate if possible Wound Debridement may be needed

Answer 234

Bacterial Sinusitis (Frontal Headache- worse when bending over, Fever, Excessive Mucous)

Answer 235

Orthostatic Proteinuria (most common cause) - generally benign and requires no intervention, presents in adolescence - repeat dipstick again in 6 hours if TRACE of protein, OR protein:creatinine ratio if POSITIVE to confirm this Transient Proteinuria - Children are found to have it incidentally - commonest cause are recurrent infections and seizures (so think this BEFORE Minimal Change Disease) - usually Benign and Self-Limiting as long as the Precipitant is treated Nephrotic Syndrome- - If 3+ Proteinuria in Dipstick - Usually due to Minimal Change Disease (look for Hypertension and Puffy Eyes (or other peripheral oedema) Type 1 Diabetes Mellitus- - If poorly controlled diabetes- leads to Diabetic Glomerulopathy - Good control of diabetes can limit this

Answer 236

2 separate urine samples should be sent for Protein:Creatinine Ratios If it is only a trace, then repeat a DIPSTICK in 6 hours They should also have Blood Pressure measured

Answer 237

It is a condition caused by Hypertrophy of the Pyloric Sphincter It leads to VOMITING after feeding, such as PROJECTILE VOMITING It may increase with Intensity as the obstruction becomes more severe There will be a SMOOTH PALPABLE OLIVE SIZED MASS especially during feeding It is diagnosed through ABDOMINAL ULTRASOUND They will have Hypokalaemic, Hypocholoraemic Metabolic Alkalosis

Answer 238

Surgical- Laparoscopic Pylorotomy Until then they should be Nil-by-mouth and kept on IV Fluids They may need resuscitation if Severe Dehydration

Answer 239

Septicaemia (or other causes of a Purpuric Rash) Slapped Cheek Syndrome Hand, Foot and Mouth Disease Scarlet Fever Measles Urticaria (Hives) Chickenpox Roseola Rubella

Answer 240

(Patient is rapidly deteriorating after a recent surgery/ infection with multiple signs below) Suspect if there is a RAPIDLY-DEVELOPING, NON-BLANCHING PURPURIC RASH which occurs AFTER any of the following: - Lethargy - Headache - Fever - Rigors - Vomiting Management- - Cultures and IV Antibiotics ASAP (Septicaemia therefore CULTURE and Abx ASAP) - Inform Senior Paediatrician - IM Benzylpenicillin if Meningitis is suspected

Answer 241

Trauma Liver Disease Drugs (Anticoagulants) Coagulopathy Thrombocytopaenia Disseminated Intravascular Coagulation Vasculitis Malignancy

Answer 242

Blisters in the hands and feet and Grey Ulcerations in the mouth It is preceded by 1 day of Fever and Lethargy It is caused by Coxsackie Virus A16 It resolves within 1 week and children do not need to be isolated

Answer 243

A Sandpaper Coarse Red Rash on the Cheeks with a Bright Red Tongue Other signs- (Scarlet H. Fever)- In addition to the TONGUE and the RASH - Sore Throat - Headache - Fever (<5 days- otherwise consider kawasaki) It is caused by Streptococcus It is managed with 10 days of Phenoxymethylpenicillin Children remain infectious until 1 day after first Antibiotics dose

Answer 244

Raised, Itchy Red Rashes that are NOT accompanied by a Fever. Can present as part of an Anaphylaxis reaction Antihistamines and Maybe Steroids can be given but it is usually self limiting

Answer 245

- Signs of Dehydration (Sunken Eyes, Drowsy, Floppy, Dry Mucous Membranes) - Projectile Vomiting- Pyloric Stenosis - Bile-stained (green) and Abdominal Distension- Intestinal Obstruction - Bloody Stool with Vomiting- Dysentery, Intussusception - Paroxysmal Coughing up to the point of vomiting- Whooping cough - Seizures/ Bulging Anterior Fontanelle- Raised Intracranial Pressure

Answer 246

It is a Neurological Condition associated with Aspirin Use while the Child has a Viral Infection So Aspirin is Contraindicated in Children It is confirmed by Liver Biopsy (Steatosis) It presents as Abnormal LFTs, Vomiting and Encephalopathy (Slurred Speech, Coma, Lethargy, Death) BTW- management is just supportive- Fluids and Electrolytes

Answer 247

It is Vitamin D Deficiency in Children (Osteomalacia in Adults) (Especially seen in children with high skin pigmentation living in low sunlight areas) Signs - Aching bones and joints - Poor growth and development - Delayed Dentition (No teeth have come through by 18 months) - Weakness and Constipation (as calcium is needed by muscles in gut and limbs)

Answer 248

Diagnosis- Low level of Blood Vitamin D and additional Radiological evidence such as Bowed Femurs and Widened Epiphyseal Plates, also Harrison's Sulci Managed- Oral Vitamin D supplements

Answer 249

GI Causes- - Acute Appendicitis - Mesenteric Adenitis - Inflamed Meckel's Diverticulum - Diverticulitis (although this is more common on the left) - Inflammatory Bowel Disease (Crohn's) Gynaecology Causes- - Ectopic Pregnancy - Ovarian Torsion - Ovarian Cyst Rupture - Pelvic Inflammatory Disease Urological Causes- - Pyelonephritis - Ureteric Colic

Answer 250

It is caused by Human Herpes Virus 6 Presentation- - Children are initially Febrile and Lethargic like HFM Disease for up to 5 days. Fevers can be as high as 40 degrees C - There is also a BLANCHING, ROSE-PINK MACULAR RASH which typically covers the trunk. This can also spread to the face and limbs. TRUNK FIRST Management is supportive

Answer 251

It is caused by a virus transmitted by Aerosols It presents as non-specific signs such as Fever, Coryza, Arthralgia and a Rash (F.A.C (E=Ear lymph nodes))- which starts on the FACE and then moves to the TRUNK (usually sparing the limbs). There is also Post-Auricular Lymphadenopathy. Measles involves the limbs MACULOPAPULAR RASH STARTING AT THE FACE Diagnosis is Serological Testing and Management is Supportive

Answer 252

It occurs in developing countries usually due to contaminated waters It presents as a Long Febrile Condition in a patient who has returned from a developing country (usually the Indian Subcontinent) Symptoms- C - Constipation then Diarrhoea H - Hepatosplenomegaly and Abdominal Pain A - Anorexia and Lethargy R - Rose Spot Rash A - Associated with Salmonella Typhi D - Dull frontal headache O - Other systemic symptoms

Answer 253

WCC is normal or Low Blood and Stool Cultures are NEEDED to confirm diagnosis Management- Ciprofloxacin or Cephalosporin is usually first line

Answer 254

Sepsis Intestinal Perforation and Haemorrhage (Typhoid damages intestines) Focal Infections in Other Sites (Osteomyelitis)

Answer 255

Focal Seizures- Visual Phenomena (Occipital Lobe Focal Seizure), Strange Smells (Temporal Lobe Focal Seizures) Generalised Seizures- Tonic (muscles stiffening), Tonic-Clonic (muscles stiffen then jerk), Myoclonic (brief jerking movements), Atonic (children fall to the floor) or Absence (unresponsive episodes where the child appears to be Daydreaming) Diagnosed with EEG. CT and MRI should be used only to find structural abnormalities. Management- - MDT input for Antiepileptics and Emergency Seizure management plans

Answer 256

They happen in 6 month to 6 year olds They are diagnosed from history alone They are managed with antiepileptics and the source of the infection is also managed

Answer 257

Seizures consisting of Myoclonic (Jack Knife Jerking) that occurs in clusters They are diagnosed with history and EEG findings of HYPSARRHYTHMIA It is associated with Developmental Regression

Answer 258

They affect children between 3-10 years old and almost always occur when they are sleeping Tonic Seizure overnight- parents sometimes find the child has fallen out of bed in the morning with messy sheets Diagnosis- History and EEG findings of CENTRO-TEMPORAL SPIKES during sleep Most children outgrow the condition during puberty

Answer 259

Remember that!

Answer 260

Lethargy and Poor Social Interaction Generally feeling unwell Altered Consciousness Continuous Crying Pale or Mottled Skin Poor Feeding and Decreased Urine Output Cool Peripheries Increased Respiratory Rate and Effort Bradycardia or Tachycardia Dehydration Fever (may not always be there)

Answer 261

Cyanosis Bradycardia or Tachycardia Mottled Skin A Non-Blanching Rash Bradypnoea or Tachypnoea Child does not Stay awake

Answer 262

FBC CRP U&Es Blood Cultures and Cultures of other tissues where necessary Creatinine Clotting Screen Lactate Blood Gas To find the source of infection- - Urinalysis - Chest Xray - Abdomen and Pelvic Xray - Lumbar Puncture if there are no contraindications (Raised ICP, Focal Neurological Signs or Fluctuating Consciousness) - Swabs

Answer 263

Broad Spectrum Antibiotics (TAZOCIN) ASAP Oxygen and Fluids if Necessary Escalate to a Senior Paediatrician within 1 hour for Review Close Monitoring They may need to Move to HDU/ PICU and may need INOTROPIC SUPPORT

Answer 264

Procalcitonin

Answer 265

It is a Bacterial or Fungal Infection of the bone which can be Acute or Chronic The most common underlying pathogens are Staph Aureus and Coagulase Negative Staphs Usually occurs due to risk factors such as Diabetes and Peripheral Vascular Diseases (also Malnutrition, Malignancy and Immunosuppression) It can occur if an infection is inoculated into the bone due to a Wound Contamination during a Surgery or Trauma

Answer 266

Acute- (signs of inflammation and a fever) - Fever - Pain - Swelling - Erythema Chronic- - Long History of Pain - A Wound or Sinus Tract that is Persistently Draining and Soft Tissue Damage - Risk Factors such as Diabetes and Peripheral Vascular Disease increase the likelihood of these occurring

Answer 267

For Definitive Diagnosis- Bone Biopsy needed for Pathology and culture Other tests to aid diagnosis- - Blood Inflammatory Markers - Imaging- 1) MRI- This is the imaging Modality of Choice 2) X ray (may be negative early on as it takes 7 days for the Periosteal Reaction to be seen, and bone necrosis is see after 10 days). 3) CT- Helps identify Necrotic Tissue and good for guiding the biopsy Other tests= Blood Cultures and Culture any Expressed Pus (Aside from the ones from Sinus Tracts)

Answer 268

Antibiotics for 4-6 weeks - Flucoxacillin plus Fusidic Acid/ Rifampicin, or Vancomycin if MRSA is suspected - If they are Penicillin-allergic then give them Clindamycin - Start with IV Antibiotics and THEN Switch to Oral Antibiotics when they are stable or 2 weeks post surgery - If it is Chronic Osteomyelitis- then delay treatment until Cultures have been taken Surgical Debridement- - This is the definitive treatment if Chronic - If Acute, Extensive Surgical Cleaning can be given early on with the Antibiotics

Answer 269

A height that is <2.5th Percentile or >2 standard deviations below their mid-parental height - Familial Causes - Constitutional Delay in Growth and Puberty - Syndromes (Down's and Turner Syndrome) - Skeletal Dysplasia (Achondroplasia (Dwarfism) and Hypochondroplasia)

Answer 270

- Malnutrition - Neglect - Chronic Conditions (Heart Failure, Coeliac Disease, Sickle Cell, Cystic Fibrosis, IBD, Renal Failure, Inflammatory Conditions) - Endocrine (Hypothyroidism, Cushing's, Growth Hormone Deficiency)

Answer 271

Shield-Shaped Chest Low Posterior Hairline Amenorrhoea Webbed Neck Short Stature

Answer 272

Sickle Cells sequester (hide away) in the Spleen and undergo Phagocytosis which leads to Extravascular Haemolysis. This leads to Spleen Congestion and Splenomegaly. The spleen is needed for Phagocytosis and this becomes compromised so the patient is at risk of infections

Answer 273

Vaso-Occlusive Crises (pain in bones, joints and abdomen)- - Microvascular Obstruction due to the Sickle Cells- could be triggered by Local Hypoxia (Like Cold Weather) - Pain and ischaemia of downstream tissues Acute Chest Crises- - The most dangerous presentations - Tachypnoea - Wheeze - Cough - Hypoxia - Pulmonary Infiltrates

Answer 274

In Crisis- - Oxygen, Fluids and Analgesia (IV Opiates) - Treat any suspected infections - Spirometry and Physiotherapy if Acute Chest Syndrome- to prevent Atelectasis (Collapse or incomplete inflation of Lung) Chronic Sickle Cell Disease- - Hydroxycarbamide if frequent crises- which Increases Fetal Haemoglobin Concentration - Top-up Transfusions, Folic Acid and Iron Chelations - Regular Exchange transfusions may be needed if Severe Anaemia - If Splenic Infarction and Immunocompromise as a result- Vaccinations (Influenza and Pneumococcal) and Antibiotics (Penicillin)

Answer 275

M - Microcytic anemia with haemolysis (reticulocytosis and unconjugated hyperbilirubinaemia) B3 - Blood film: sickle cells, target cells, reticulocytosis with polychromasia H2 - Hyposplenism signs: Howell-Jolly bodies and nucleated red blood cells D - Definitive diagnosis: haemoglobin electrophoresis and genetic testing

Answer 276

Sequestration Crisis (Splenomegaly, Pale Skin, Headache, Fatigue)- as oxygen flow is low to organs and splenomegaly compresses LUQ which can lead to a headache Osteomyelitis Stroke Pulmonary Fibrosis and Pulmonary Hypertension Aplastic Crisis (Red Cell Aplasia) (Low red cells which leads to anaemia)- If tachycardia and tachypnoea and no jaundice Gallstones Iron Overload Chronic Kidney Disease

Answer 277

It is the most common hip disorder in adolescents (10-16 year olds). It occurs when weakness in the proximal femoral growth plate allows Displacement of the Capital Femoral Epiphysis - Obese, Adolescent Males - Endocrine Disorders (Hypothyrodism and Hypogonadism) - Afro-Caribbean or Hispanic Ethnicity Signs- - Hip pain and a limp (It can be acute or chronic) - Pain may be referred to the KNEE - Reduced range of movement of Hip Flexion- and AFFECTED LEG may appear shorter - Positive Trendelenburg Sign (Pelvis opposite affected leg drops when the unaffected leg is lifted)

Answer 278

Diagnosed- Anterolateral and Frog-Leg Xrays which may show Short-Displaced Epiphysis and Widened Growth Plate (Ice cream falling off cone) Management- Surgical management- Fixation with a Screw. Prompt treatment is needed to avoid Blood Flow being Disrupted and reduce the risk of Avascular Necrosis of the Femoral Head

Answer 279

- Roll on side (if safe to do so) and give OXYGEN and GLUCOSE - First line Treatment if seizure has not resolved within 5 minutes= IV Lorazepam or Buccal Midazolam or Rectal Diazepam - If Seizures continue after 10 minutes- IV Lorazepam - If they continue for another 10 minutes- IV Lorazepam (consider anaesthesia and call for senior support) - IV Phenytoin (IV Phenobarbitane if they Normally take Phenytoin) - Rapid Sequence Induction with Sodium Thiopentane

Answer 280

Focal Neurological Deficit Memory Loss Behavioural Problems Hypoxic Brain Injury

Answer 281

It is a hernia that cuts off the blood supply to the intestines and tissue in the abdomen - Abdominal pain and vomiting - They can have an intermittent history of pain. The bowel that forms the hernia can become ischaemic and necrotic which leads to Sepsis or Perforations Management- - Surgery and follow up with a Mesh to strengthen the site

Answer 282

Croup (temperature usually not seen) - Cough and Coryzal symptoms - Symptoms are worse at Night Epiglottitis - High Temperature, Struggling to Breathe and Drooling - Very irritable Bacterial Tracheitis - Intermediary symptoms between Croup and Epiglottitis - Shortness of Breath and Temperature

Answer 283

A sudden-onset severe pain in one testicle- often following a minor trauma It will be high-riding in the scrotum Unilateral loss of Cremasteric Reflex Negative Prehn's Sign (No relief on lifting the testicle) Management- Orchidopexy. BOTH testicles should be fixed to prevent the other one undergoing torsion as well

Answer 284

It is a remnant of the Mullerian Duct It causes less pain than Torsion and here may be a tiny blue dot visible

Answer 285

It is a Cyanotic Heart Disease consisting of 4 parts and 1 extra part - Ventricular Septal Defect - Overriding Aorta (An Aorta that overrides the right and the left ventricle as a result of the Ventral Septal Defect) - Right Ventricular Hypertrophy - Right Ventricular Outflow Tract Obstruction due to Pulmonary Stenosis= the main cause of the Cyanosis

Answer 286

It is diagnosed Antenatally (Boot Shaped Heart on ECHO) or on the detection of a Murmur within the first few months of life - Murmur - Cyanosis - Weakness or Fatigue during feeding or physical activity - Tet Spells= Acute Episodes of Cyanosis (there is Reversal of the shunt across the VSD leading to a Right-to-Left Shunt and Worsening Cyanosis) Management- - Tet Spell= 1) Emergency- Lie the Baby on their back and bend their knees 2) Give them Oxygen as well 3) A Vasoconstrictive Agent (Phenylephrine) is reserved for Severe Episodes 3) Prophylaxis- Propanolol - General Tetralogy of Fallot= 1) Surgery- Fixing the Ventricular Septal Defect and Right Ventricular Outflow Tract Obstruction is Definitive

Answer 287

Strep Pneumoniae for Bacterial and EBV for Viral Bacterial- Cervical Lymphadenopathy Viral- Headache, Apathy, Abdominal Pain Also the CENTOR Criteria gives the likelihood of the infection being bacterial

Answer 288

1) Tonsilar Exudate 2) Tender Anterior Cervical Lymphadenopathy 3) Fever over 38 4) Absence of Cough

Answer 289

If CENTOR = 3 or 4 or if they are Immunosuppressed or Systemically Upset 1) Penicillin V 500mg PO QDS for 5-10 days 2) Otherwise Clarithromycin/ Erythromycin 250-500mg PO BD for 5 days Tonsilectomy IF- - Sore Throats are due to Tonsilitis - Person has at least 5 episodes per year - Symptoms have been occurring for at least a year - The episodes prevent normal functioning

Answer 290

3-10 year olds It is a benign cause of LIMPING due to inflammation of the Synovial Lining It is caused by a Viral Infection (usually an Upper Respiratory Tract Infection) that happens before the limp Signs- - Pain in Hip and Knee (referred from hip) - Low Grade Fever - Similar to Septic Arthritis but Transient Synovitis is more MILD (Milder pain and Milder pain)

Answer 291

Blood tests- High WCC and Inflammatory markers point to Septic Arthritis but may be Normal or Raised inboth conditions Ultrasound may show Joint Effusion but may be normal If unsure whether it is Septic Arthritis or Transient Synovitis- do Joint Aspiration (with Ultrasound Guidance and Cultures) Culture- If Septic Arthritis= Aspiration shows Bacteria within the joint space MANAGEMENT- - Supportive treatment as it resolves in 7 days

Answer 292

Fever> 38C Refusal to weight bear on affected side (so the pain is SUPER BAD) Raised Inflammatory Markers (ESR and CRP) Raised WCC

Answer 293

It is a PULMONARY OEDEMA that occurs due to the delayed clearance of Foetal Alveolar Fluid. It is the MOST COMMON cause of respiratory distress in newborns It usually occurs after C Section as passage through the birth canal usually puts pressure on the baby thorax to help them expel the fluid Signs- - Respiratory Distress= Tachypnoea, Laboured breathing and Cyanosis/ Pale

Answer 294

Diagnosis- Clinical Diagnosis and Chest X ray shows HYPERINFLATED LUNGS and FLUID LEVEL Management- Oxygen Support and TTN should resolve on its own on the first 3 days of life

Answer 295

It is a Cyanotic Heart Condition where the origins of the Aorta and the Pulmonary Artery are swapped Usually in the babies of Diabetic Mother Diagnosis- Most are made Antenatally, Some may be diagnosed Postnatally with Cyanosis in the first 24 hours of life and a LOUD S2 Management- Surgery is needed ASAP. Give them PROSTAGLANDIN E (Alprostadil) to keep the Ductus Arteriosus open until they get the surgery

Answer 296

Patau, Edward and Down Syndrome Remember Patau= Puberty (13). Edward= Education (18), Down= Degree (21)

Answer 297

Patau- They rarely survive more than a few weeks - Holoprosencephaly (failure of the 2 cerebral hemispheres to divide so may only have ONE EYE and ONE NOSTRIL) - Cleft lip and palate - Microcephaly - Polydactyl - Congenital Heart Disease - Rocked Bottomed feet (Clubbed Feet) (seen in Edward Syndrome as well) - Outer fingers overlap the inner fingers Edward- they rarely survive more than a few months - Low set ears - Micrognathia - Microcephaly - Overlapping 4th and 5th fingers - Congenital Heart Disease

Answer 298

It is an Autosomal Dominant, Neurocutaneous Syndrome consisting of Cellular Hyperplasia, Tissue Dysplasia and Hamartomas involving multiple organs Signs- - Spasms (repetitive behaviours) with Hypsarrhythmias seen on EEG - Ash-leaf Macules (Hypopigmentation), Shagreen Patches (leathery patches) , Facial Angiofibromas, Subungal Fibromas (Skin) - Subependymal nodes, Seizures and Learning Disability (Neurological/ Cognitive) - Renal- Angiomyolipomas - Cardiac- Cardiac Rhabdomyomas Management- - Angiofibromas<2mm= LASER - Angiofibromas>2mm= Dermabrasion or Surgical Resection - If Renal disease, they may require Antihypertensive therapy - Tubers should be regularly imaged and may require RESECTION

Answer 299

It is caused by an XO Karyotype and it affects ONLY FEMALES as a result of this Signs- - Delayed Puberty and Short Stature - Lymphoedema of hands and feet in neonate - Spoon-shaped nails - Webbed neck - Wide spaced Nipples (Shield Shaped Chest) - Hypothyroidism - Recurrent Otitis Media - Congenital Heart Defect (Bicuspid Aortic Valve Usually so Aortic Stenosis, but can also be Coarctation) Managed with Growth Hormone therapy and Oestrogen Replacement to allow the Secondary Sexual Characteristics to develop

Answer 300

Polyuria Polydipsia Weight Loss Can lead to DKA so may present with this Diagnosis- - Raised Blood Glucose (>11), High HbA1c, Ketones and Fasting Glucose (>7) Management- - Insulin Replacement (short acting- after meals and long acting-at night)

Answer 301

Insulin requirements may be low if the pancreas is still able to produce a significant amount of insulin. This is the Honeymoon period until all the beta cells are destroyed

Answer 302

Delayed Puberty and Obesity Hypertension Renal Disease Retinopathy Associated with Thyroid Disease (most associated with this) and Coeliac Disease

Answer 303

Signs (REMEMBER THE FIRST 4) - Fever - Vomiting - Poor Feeding - Lethargy - Irritability - Failure to Thrive - Offensive Urine - Abdominal Pain - Dysuria Diagnosis- Positive Leucocytes and Nitrites and Positive Urine Cultures

Answer 304

Oral Antibiotics (Lower UTI= Nitrofurantoin and Upper UTI= Cephalosporin) Conduct pregnancy test if necessary

Answer 305

Poor Urine Flow Abdominal or Bladder mass Raised Creatinine Infection with non-E.Coli organisms Septicaemia Failure to respond to treatment within 2 days

Answer 306

Ultrasound- for structural abnormalities, only needed if Atypical UTI DMSA (Scintigraphy)- for Scarring- don't do until 4 months after UTI MCUG- check for abnormal bladder function

Answer 307

Urine Collection Pad

Answer 308

Common- - Tenderness and aches around site of injection - Fever and unwell for few hours - Fever is common with Meningococcal vaccination so should give them Prophylactic Paracetamol and parents should be warned Rare- - Anaphylaxis - Rotavirus vaccination can cause Intusussception - MMR can cause Seizures and Idiopathic Thrombocytopaenia Purpura

Answer 309

Egg Allergy - Avoid Yellow Fever vaccination - Avoid Influenza if they have been admitted to PICU because of their egg allergy Previous anaphylaxis to vaccine components Immunosuppression- should not have live attenuated vaccinations Intussusception- should not have Rotaviruses

Answer 310

They are the MOST COMMON CONGENITAL CARDIAC ABNORMALITY Signs- (Effort when breathing and murmur) - Asymptomatic if small - Pan-Systolic Murmur - If Large, Shortness of Breath on exertion (such as when breastfeeding) - As breathing is more effort, they GROW LESS - Heart Failure Diagnosis- - ECHOCARDIOGRAM - CXR and ECG show Enlarged Left Ventricle Management- - Majority of them self-resolve so just observe calorie intake and follow up - If they don't resolve, close them via catheter intervention

Answer 311

It is the abnormal flow of Urine from the Bladder into the Upper Urinary Tract due to either a Short or Absent Valve. Backflow of urine basically It is diagnosed through MCUG 2 main risk factors- - Neurogenic Bladder - Posterior Urethral Valves (only occurs in males) It presents as: - RECURRENT UTIS (if this happens in general, FIND OUT WHY via US/ if<6 months MCUG) - Incomplete Voiding

Answer 312

Grade 1- into Ureters only Grade 2- into Pelvis causing no dilatation of Ureters Grade 3- into Pelvis causing Mild Dilatation Grade 4- into pelvis causing Moderate Dilatation Grade 5- through to Calyces Management- - Grades 1-3 resolve on their own - Surgery may be needed if Grade 4 or 5 or if Scarring, Sepsis, CKD, Reflux Nephropathy

Answer 313

Oculocutaneous Albinism Retinoblastoma Retinopathy of Prematurity Cataracts Juvenile Idiopathic Arthritis

Answer 314

Autosomal Recessive Poor Development in the Fovea of an Albino patient Care should be taken to avoid further damage

Answer 315

It is a tumour of the retina that occurs in Infancy or Childhood Children present with White Eye Reflex (basically no Red Eye Reflex) It occurs due to a Double Hit of the Rb Suppressor Gene

Answer 316

It is a visual impairment in Premature Babies It is thought to be due to Free Radicals released by the Supplemental Oxygen Therapy which leads to Retinal Fibrosis, Retinal Detachment and then Blindness Babies born <32 weeks gestation or <1.5kg should be screened for Retinopathy of Prematurity via Fundoscopy Treatment- Laser Therapy

Answer 317

They are a cause of Absent Red Reflex, typically caused by Infections or Metabolic Defects

Answer 318

Through Chronic Anterior Uveitis- so Screen for this in Juvenile Idiopathic Arthritis

Answer 319

It occurs especially if the babies are exclusively Breastfed Babies BRUISE more easily and there may be INTERNAL BLEEDING which can lead to Jaundice Management- - Subcutaneous Vitamin K injections to prevent Haemorrhagic Disease of the Newborn - Can also be Oral If Unmanaged it can lead to Haemorrhagic Shock

Answer 320

It is the Most Common Abdominal Tumour in Children occurring in 2-5 year olds It presents as: - An Abdominal mass that does NOT cross the midline - Abdominal Distension - Haematuria - Hypertension - Although it is usually Asymptomatic

Answer 321

Diagnosis- - CTAP identifies extent and spread of disease - DEFINITIVE DIAGNOSIS- Renal Biopsy which shows Small Round Blue Cells Management- - Nephrectomy, Chemotherapy, Radiotherapy

Answer 322

It can be used by women who can not use Oestrogen Women who have contraindications such as >15 cigarettes, >35 years old and migraine It can also be used by Breastfeeding Women

Answer 323

Meiotic Dysjunction (94% of cases) - Error during meiosis Robertsonian Translocation (5% of cases) - A piece of chromosome 21 or a complete copy of it are attached to another chromosome (usually 14) Mosaicism (rare) - some cells have trisomy 21 while others do not

Answer 324

Croup= STEEPLE ANGLE (subglottic narrowing) Acute Epiglottitis= THUMB SIGN