Paediatrics Flashcards

Question

What is presentation of eczema?

Answer 1

Itchy, dry inflammatory skin disease.

Answer 2

Atopic – ‘genetic barrier dysfunction’ Seborrheoic – face/scalp – scale associated Discoid – annular/circular patches Pomphylx – vesicles affecting palms/soles Varicose – oedema/venous insufficiency

Answer 3

Exogenous (external cause) Allergic contact dermatitis (sensitised to allergen) Irritant contact dermatitis (friction, cold, chemicals e.g acids,alkalis, detergents, solvents) Photosensitive/photoaggravated eczema

Answer 4

* Flares can be associated with: * Infections/viral illness * Environment: central heating, cold air * Pets: if sensitised/allergic * Teething * Stress * Sometimes no cause for flare found

Answer 5

Loss of water Irritants may penetrate (soap detergeant, solvents, dirt) Allergens may penetrate (pollens, dust-mite antigens, microbes)

Answer 6

Mainly the scalp and the face

Answer 7

Babies under 3 months, usually resolves by 12 months

Answer 8

Associated with proliferation of various species of the skin commensal Malassezia in its yeast form

Answer 9

•Emollients, antifungal creams, antifungal shampoos, mild topical steroids

Answer 10

* Associated with oedema, varicose veins, chronic leg swelling * Skin often dry and inflamed * Skin may ulcerate

Answer 11

Emollients, topical steroids, compression stockings

Answer 12

* Immediate reactions (lip swelling, facial redness/itching, anaphylactoid symptoms) * Late reactions (worsening of eczema 24/48 hours after ingestion) – especially if pattern with specific food (food diaries encouraged). * GI problems * Failure to thrive * Severe eczema unresponsive to treatment * Severe generalised itching – even when the skin appears clear * Atopy can be associated with food allergy

Answer 13

* 2 ways to test for food allergy: * Blood test for specific IgE antibodies to certain foods * Skin prick testing

Answer 14

•Commonest: milk/dairy, soy, peanuts, eggs, wheat, fish

Answer 15

•Airborne allergens (can also be tested for in same way) - house dust mite, pet dander, pollens

Answer 16

* Emollients (Lotions, creams or ointments – fragrance free, greasier ointments more effective) * Topical steroids * Calcineurin inhibitors (e.g protopic – steroid sparing topical agents) * UVB light therapy * Immunosuppressive medication

Answer 17

* Very potent (Dermovate)600x * Potent (Betnovate)100x * Moderate (Eumovate) 25x * Mild (Hydrocortisone)

Answer 18

Pustules and honey coloured crusted erosions

Answer 19

Staph aureus

Answer 20

Topical antibacterial (fucidin) Oral antibiotic (flucloxacillin)

Answer 21

Pearly papules, umbilicated centre

Answer 22

Molluscipox virus

Answer 23

Treatment is not usually recommended. If lesions are troublesome or considered unsightly, use simple trauma or cryotherapy, depending on the parents' wishes and the child's age: ## Footnote Squeezing (with fingernails) or piercing (orange stick) lesions may be tried, following a bath. Treatment should be limited to a few lesions at one time Cryotherapy may be used in older children or adults, if the healthcare professional is experienced in the procedure Eczema or inflammation can develop around lesions prior to resolution. Treatment may be required if: → Itching is problematic; prescribe an emollient and a mild topical corticosteroid (e.g. hydrocortisone 1%) → The skin looks infected (e.g. oedema, crusting); prescribe a topical antibiotic (e.g. fusidic acid 2%)

Answer 24

SKin coloured

Answer 25

* Common non-cancerous growths of the skin caused by infection with human papillomavirus (HPV) * Sole foot – verruca * Transmitted by direct skin contact

Answer 26

* Chicken pox * Measles * Rubella * Roseola (herpes virus 6) * Erythema infectiosum (Parvovirus B19, slapped cheek )

Answer 27

* Associated viral illnesses * Common * Fever, malaise, headache

Answer 28

•Either reaction to a toxin produced by the organism, damage to the skin by the organism, or an immune response.

Answer 29

* One infection is thought to confer lifelong immunity. * Immunocompromised individuals are susceptible to the virus at all times.

Answer 30

* Red papules (small bumps) progressing to vesicles (blisters) often start on the trunk. * Itchy. Associated with viral symptoms

Answer 31

10-21 days

Answer 32

1-2 days before rash appears and until lesions have crusted

Answer 33

* Viral symptoms. * Erythematous rash cheeks initially and then also lace like network rash (trunk and limbs). Can take 6w to full fade.

Answer 34

* Very rarely…. * Aplastic crisis (if haemolytic disorders) * Risk to pregnant women (spontaneous abortion, intrauterine death, hydrops fetalis)

Answer 35

* Usually Coxsackie virus A16 * (can also be due to Enterovirus 71 and other coxsackivirus types)

Answer 36

•Blisters on the hands, feet and in the mouth. Viral symptoms.

Answer 37

Lip swelling and fissuring Oral mucosal lesions: ulcers and tages, cobblestone appearance Crohn's disease

Answer 38

* Painful, erythematous subcutaneous nodules * Over Shins; sometimes other sites •Slow resolution - like bruise, 6-8 weeks

Answer 39

* Infections – Streptococcus, Upper respiratory tract * Inflammatory bowel disease * Sarcoidosis * Drugs – OCP, Sulphonamides, Penicillin * Mycobacterial Infections * Idiopathic

Answer 40

Coeliacs disease

Answer 41

* Rare but persistent immunobullous disease that has been linked to coeliac disease * Itchy blisters can appear in clusters * Often symmetry * Scalp, shoulders, buttocks, elbows and knees

Answer 42

* Detailed history * Coeliac screening * Skin biopsy

Answer 43

•Emollients, gluten free diet, topical steroids, dapsone

Answer 44

* Wheals/hives * Associated angioedema (10%) * Areas of rash can last from few minutes up to 24 hours

Answer 45

Acute is less than 6 weeks Chronic is more than 6 weeks

Answer 46

* Many causes: * Viral infection * Bacterial infection * Food or drug allergy * NSAIDS, OPIATES, * Vaccinations * Chronic urticaria – idiopathic often no cause found. Likely autoimmune cause.

Answer 47

* Antihistamines * Newer generation e.g desloratadine * 3 x daily (off licence doses) * Ranitidine * Montelukast * Omalizumab * Ciclosporin

Answer 48

Genetic susceptibility (6-10% of all CHD have underlying chromosomal problems) Teratogenic insult (18-60 days post ceonception) Environmental factors( drgus such as alcohol cocaine, infections such as TORCH and others, maternal factors such as diabetesm SLE)

Answer 49

VSD and ASD

Answer 50

multiple cardiac problems may be present endocardial cushion defect (most common, 40%, also known as atrioventricular septal canal defects) ventricular septal defect (c. 30%) secundum atrial septal defect (c. 10%) tetralogy of Fallot (c. 5%) isolated patent ductus arteriosus (c. 5%)

Answer 51

Turner = co-arctation of the aorta Noonan = Pulmonary stenosis Williams = supravalvular AS

Answer 52

Age 2-7 years Soft systolic; vibratory, musical,”twangy” Apex, left sternal border **Increases in supine position and with exercise**

Answer 53

Age 8-10 years Soft systolic; vibratory Upper left sternal border, well localised, not radiating to back Increases in supine position, with exercise Often children with narrow chest

Answer 54

Age 2-10 years 1/6-2/6 systolic; harsh Supraclavicular, radiates to neck Increases with exercise, decreases on turning head or extending neck

Answer 55

Age 3-8 years Soft, indistinct Continuous murmur, sometimes with diastolic accentuation Supraclavicular Only in upright position, disappears on lying down or when turning head

Answer 56

3 main types : - subaortic - perimembranous - muscular L to R shunt

Answer 57

Pansystolic murmur lower left sternal edge, sometimes with thrill In very small VSDs, early systolic murmur In very large VSDs diastolic rumble due to relative mitral stenosis Signs of cardiac failure in large VSDs, eventually leading to biventricular hypertrophy and pulmonary hypertension

Answer 58

Eisenmenger syndrome occurs when the increased pressure of the blood flow in the lung becomes so great that the direction of blood flow through the shunt reverses. Oxygen-poor (blue) blood from the right side of the heart flows into the left side of the heart and is pumped to your body so you don't receive enough oxygen to all your organs and tissues. Results from intracardiac communication and causes cyanosis, pulmonary hypertensio and shunt reversal. Erythrocytosis also follows

Answer 59

Amplatzer device Patch closure

Answer 60

Singular AV valve with ostium primum ASD and high VSD

Answer 61

Asymptomatic in mild stenosis, in moderate and severe exertional dyspnoea and fatigue Ejection systolic murmur upper left sternal border with radiation to back

Answer 62

Mostly asymptomatic, if severe, reduced exercise tolerance, exertional chest pain, syncope Ejection systolic murmur upper right sternal border, radiation into carotids

Answer 63

Pulmonary Vascular Resistance Falls Pulmonary Blood Flow Rises Systemic Vascular Resistance is increased Ductus Arteriosus Closes Foramen Ovale Closes Ductus Venosus Closes

Answer 64

fluid restriction/ diuretics, prostaglandin inhibitors (Indomethacin, Ibuprofen), surgical ligation In term babies good chance of spontaneous closure, not prostaglandin sensitive

Answer 65

Re-open PDA with Prostaglandin E1 or E2 Resection with end-to-end anastomosis Subclavian patch repair Balloon Aortoplasty

Answer 66

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

Answer 67

cyanosis causes a right-to-left shunt ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually cause a murmur) a right-sided aortic arch is seen in 25% of patients chest x-ray shows a 'boot-shaped' heart, ECG shows right ventricular hypertrophy

Answer 68

surgical repair is often undertaken in two parts cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm

Answer 69

Elevated in hepatocellular damage (hepatitis)

Answer 70

Elevated in biliary disease

Answer 71

•Coagulation * •Prothrombin time (PT)/INR * •APTT * Albumin * Bilirubin * (Blood glucose) * (Ammonia)

Answer 72

Discolouration of the skin due to accumulatino of bilirubin

Answer 73

•Usually visible when total bilirubin \>40-50 umol/l

Answer 74

**_•Early (\<24 hours old)_** * Always pathological * Causes: Haemolysis, Sepsis **_•Intermediate (24hrs – 2 weeks)_** •Causes: Physiological, Breast milk, Sepsis, Haemolysis **_•Prolonged (\>2 weeks)_** •Causes: Extrahepatic obstruction, Neonatal hepatitis, Hypothyroidism, Breast milk

Answer 75

* Shorter RBC life span in infants (80-90 days) * Relative polycythaemia * Relative immaturity of liver function

Answer 76

* Unconjugated jaundice * Develops after first day of life

Answer 77

* Unconjugated jaundice * Can persist up to 12 weeks It is essentially an extension of physiological jaundice and the exact mechanism of action is unknown

Answer 78

* Sepsis * Haemolysis * •ABO incompatibility * •Rhesus disease * •Bruising/cephalhaematoma * •Red cell membrane defects (e.g. spherocytosis) * •Red cell enzyme defects (e.g. G6PD) •(Abnormal conjugation) * •Gilbert’s disease – common, mild * •Crigler-Najjar syndrome – v. rare, severe

Answer 79

* Sepsis (urine + blood cultures, TORCH screen) * Haemolysis * ABO incompatibility (Blood group, DCT) * Rhesus disease (Blood group, DCT) * Bruising/cephalhaematoma (clinical examination) * Red cell membrane defects (e.g. spherocytosis) (Blood film) * Red cell enzyme defects (e.g. G6PD) (G6PD assay) * (Abnormal conjugation) * Gilbert’s disease (genotype/phenotype) * Crigler-Najjar syndrome (genotype/phenotype)

Answer 80

Deposits of bilirubin in the brain * Unconjugated bilirubin is fat-soluble (water insoluble) so can cross blood-brain barrier * Neurotoxic and deposits in brain

Answer 81

•Early signs – encephalopathy – poor feeding, lethargy, seizures

Answer 82

•Late consequences – severe choreoathetoid cerebral palsy, learning difficulties, sensorineural deafness

Answer 83

* Treatment for unconjugated jaundice * Visible light (450nm wavelength) (not UV) converts bilirubin to water soluble isomer (photoisomerisation) * Threshold for phototherapy in infants guided by charts

Answer 84

Split bilirubin - determines if it is a prolonged / not-prolonged jaundice

Answer 85

•Biliary atresia * •Conjugated jaundice, pale stools •Choledochal cyst * •Conjugated jaundice, pale stools •Alagille syndrome * •Intrahepatic cholestasis, dysmorphism, congenital cardiac disease

Answer 86

Pale stools dark urine

Answer 87

Obstruction of extra hepatic bile ducts

Answer 88

Intraoperative cholangiogram is the gold standard for confirming obstruction **Kasai portoenterostomy** Success rate diminishes rapidly with age Best results if performed before 60 days (\<9 weeks) Potential medication - ursodeoxycholic acid

Answer 89

_•Biliary atresia_ •(split bilirubin, stool colour, ultrasound, liver biopsy) _•Choledochal cyst_ •(split bilirubin, stool colour, ultrasound) _•Alagille syndrome_ •(dysmorphism, genotype)

Answer 90

* Alpha-1-antitrypsin deficiency * Galactosaemia * Tyrosinaemia * Urea cycle defects * Haemochromatosis * Glycogen storage disorders * Hypothyroidism * Viral hepatitis * Parenteral nutrition

Answer 91

* Alpha-1-antitrypsin deficiency (phenotype/level) * Galactosaemia (GAL-1-PUT) * Tyrosinaemia (amino acid profile) * Urea cycle defects (ammonia) * Haemochromatosis (iron studies, liver biopsy) * Glycogen storage disorders (biopsy) * Hypothyroidism (TFTs) * Viral hepatitis (serology, PCR) Parenteral nutrition (history

Answer 92

Poor appetite Irritable Lack of energy Abdominal pain or distension Withholding or straining Diarrhoea (this is what the symptom they present with)

Answer 93

Poor appetite Irritable Lack of energy Abdominal pain or distension Withholding or straining Diarrhoea

Answer 94

Opiates Gaviscon

Answer 95

Social * poor diet * •Insufficient fluids * •Excessive milk * potty training / school toilet Physical * intercurrent illness * medication Family history Psychological (secondary) Organic

Answer 96

Dietary * fibre * fruit * vegetables * fluids * milk _Psychological elements of therapy:_ Reduce aversive factors Make going to the toilet a pleasant experience * Correct height * Not cold * School toilets * Soften stool and remove pain Avoid punitive behaviour from parents Reward ‘good’ behaviour General praise Star charts Soften stool and stimulate defecation: - Osmotic laxitives (lactulose) - Stimulant laxitives (senna, picolax) - Isotonic laxitives (movicol) Advantages - non-invasive, given by patients. Disadvantages - non-compliance, side effects

Answer 97

Megarectum can press on bladder and urethra - can cause poor stream, incomplete voiding and can result in UTI's

Answer 98

Empty impacted rectum Empty colon Maintain regular stool passage Slow weaning off treatment (can be movicol disimpaction) - can be messy though

Answer 99

Crohns has a massive effect of weight loss and growth failure in children. UC has more diarrhoea and rectal bleeding

Answer 100

Laboratory investigations Full blood count & ESR * Anaemia * Thrombocytosis * Raised ESR Biochemistry **•Stool calprotectin** * Raised CRP * Low Albumin Microbiology •No stool pathogens In crohn's symptoms are not very strong, you should do tests at low threshold.

Answer 101

Children = 60% Adults = 20%

Answer 102

Definitive investigations Radiology (especially Crohn’s disease) * MRI - recquires drinking a lot of water, so you can't put them under GA. Asking a child to sit still for so long is really difficult to this is probably reserved for older children * Barium meal and follow-through (younger kids) Endoscopy * Ileocolonoscopy * Upper GI endoscopy * Mucosal biopsy * Capsule endoscopy * Enteroscopy

Answer 103

Induce and maintain remission Correct nutritional deficiencies Maintain normal growth and development

Answer 104

Medical Anti-inflammatory Immuno-suppressive Biologicals ( Infliximab) Nutritional Immune modulation Nutritional supplementation Surgical Lecture notes: Crohn’s in childhood is managed by nutrition– child is given milkshakes. UC – 5 aminosalycilate, distal can give rectal preparations, usually need course of oral corticosteroids. SE = greasy hair, weight gain and spots. Azathioprine Crohn’s – chance of disease coming back is really high 5ASA’s don’t work. Put them on azathioprine from the start. Bottom-up treatment for Crohn's disease = Polymeric diet or oral prednisolone Steroid sparing agents azathioprine/6MP or methotrexate Biologicals (infliximab/adalibumab) Surgery

Answer 105

Be friendy and smile acknowledge child early on Get down to their level Utilise play Ask age appropriate questions Positioning of baby can be on parents lap

Answer 106

2 years of age

Answer 107

If there is body disproportion

Answer 108

14 years in girls 16 years in boys

Answer 109

Height, lenght, weight Growth charts and plotting MPH and target centiles Growth velocity Bone age Pubertal assessment

Answer 110

Familial Constitutional SGA/IUGR

Answer 111

Undernutrition Chronic ilness (JCA, IBD, Coeliac) Iatrogenic (steoids) Psychological and social Hormonal (GHD, hypothyroidism) Syndromes (Turner, P-W) (turner is a spectrum of dysmorphia - you need to perform a karyotype)

Answer 112

IGF-1 GH stimulation test (given arginine or insulin growth factor?)

Answer 113

Look for abnormalities of the pituitary

Answer 114

- B (breast - for girls) 1-5 • G (genital development) 1-5 • PH (pubic hair) 1 to 5 • AH (axilary hair) 1 to 3 • T (2ml to 20ml) • SO eg statement as B3 PH3 or G2 PH2 6/6

Answer 115

Boys – early \< 9 years (rare) – delayed \>14 (common, especially CDGP) • Girl – early \<8 years – delayed \>13 (rare) CDGP = constitutional delay in growth and puberty

Answer 116

Gonadal dysgenesis (Turner 45X, Klinefelter 47XXY) * Chronic disease (Crohn’s, asthma) * Impaired HPG axis (septo-optic dysplasia, craniopharyngioma, Kallman’s syndrome) * Peripheral (cryptorchidism, testicular irradiation)

Answer 117

Pubertal development – Breast development in girls – Testicular enlargement in boys * Growth spurt * Advanced bone age

Answer 118

• Need to exclude pituitary lesion---- MRI

Answer 119

Abnormal sex steroid hormone secretion * Gonadotrophin independent (low/prepubertal levels of LH and FSH) * Clinical picture: secondary sexual characteristics

Answer 120

Need to exclude congenital adrenal hyperplasia

Answer 121

Do not guess the sex of the baby! Multidisciplinary approach (paed endo, surg, neonatologist, geneticist, psychologist) Exam: gonads?/ internal organs Karyotype Exclude Congenital Adrenal Hyperplasia!- risk of adrenal crisis is first 2 weeks of life

Answer 122

– Breast budding (Tanner Stage B 2) in a girl – Testicular enlargement (Tanner Stage G2 -T 3- 4 ml) in boy

Answer 123

Athyreosis / hypoplastic / ectopic Dyshormonogenic

Answer 124

Hasimotos thyroiditis

Answer 125

Lack of eight gain Pubertal delay (or precocity) Poor school performance

Answer 126

Main ones: - Gallstones - PCOS - Obstructuve sleep apnoea - Atherosclerotic cardiocvascular disease - non-alcoholic steatohepatitis - SCFE Metabolic syndrome  Fatty liver disease (nonalcoholic steatohepatitis)  Gallstones  Reproductive dysfunction (eg, PCOS)  Nutritional deficiencies  Thromboembolic disease  Pancreatitis  Central hypoventilation  Obstructive sleep apnea  Gastroesophageal reflux disease  Orthopaedic problems (slipped capital femoral epiphysis, tibia vara)  Stress incontinence  Injuries  Psychological  Left ventricular hypertrophy  Atherosclerotic cardiovascular disease  Right-sided heart failure

Answer 127

SIMPLE OBESITY • Drugs • Syndromes (would also have learning difficulties) • Endocrine disorders (woul dhave growth failure) • Hypothalamic damage (loss of appetite control)

Answer 128

Nausea and vomitting Abdominal pain Sweet smelling breath Drowsiness Rapid deep signing respiration Coma

Answer 129

Immediately test finger prick blood capillary glucose Result \>11mmol/l - Diabetes Result \<11mmol/l - Other cause

Answer 130

Wt (kg ) = 2 x (Age +4)

Answer 131

Blood Volume (mls) = 80mls/kg

Answer 132

0.5 -1 ml/kg/hour

Answer 133

20ml/kg/day

Answer 134

80+ (2 x age)

Answer 135

1 in 5 people don't have the enzyme to metabolise codein 1 in 10-15000 have hella enzymes which makes the peak dose massive - can cause respiratory depression and even death. Codein is a prodrug so we might as well give morphine

Answer 136

20ml/kg bolus 0.9% NaCl

Answer 137

0.9% NaCl / 5% Dextrose +/- 0.15% KCl 4ml/kg 1st 10kg 2ml/kg 2nd 10 kg 1ml/kg every kg thereafter 10 yrs = 2 x (10+4) = 28kg = 40+20+8 = 68mls/hr

Answer 138

**Feed refusal** **Bile vomits** **Colour** (sick babies tend to look grey - poorly perfused skin) **Tone** (baby is normally quite active, floppy is a bad sign) **Temperature** (Low temperature is more worrying than high temperature, high temperature is often jsut normal response to virus) (green bile vomit is a bowel obstruction until proven otherwise - has to be green)

Answer 139

pain vomiting fever Classical appendicitis features tenderness over mcBurney's point.

Answer 140

McBurney's point is the name given to the point over the right side of the abdomen that is one-third of the distance from the anterior superior iliac spine to the umbilicus (navel). This point roughly corresponds to the most common location of the base of the appendix where it is attached to the cecum.

Answer 141

Abscess Mass Peritonitis

Answer 142

Pain migrates from the centre to the right iliac fossa Pain is worse on coughing or when going over speed bumps. Children can't typically hop on their right leg due to the pain

Answer 143

mild pyrexia is common - temperature is usually 37.5-38oC. Higher temperatures are more typical of conditions like mesenteric adenitis

Answer 144

anorexia is very common. It is very unusual for patients with appendicitis to be hungry

Answer 145

Raised inflammatory markers with a compatiable history To exclude pregnancy, renal colic and UTI a urine sample may also be taken, it might show mild leucocytosis but there shouldn't be any nitrites USS might be useful to exclude pelvic organ pathology, although the appendix can't always be seen.

Answer 146

Laparoscopic surgery is the treatment of choice. Open surgery is an alternative Pre-operative abx to reduce rates of wound infection Perforated appendicitis requires abdominal lavage Appendix mass is given broad spectrum abx and consideration is given to performaing an interval appendicectomy.

Answer 147

features: short duration central constant not made worse by movement no GIT disturbance no temperature site & severity of tenderness vary

Answer 148

Appendicitis Mesenteric adenits (follows from a symtoms of a cold, high temperature) Pneumonia (commonly in the right lower lobe - will have a soft abdomen, also has a high respiratory rate and a high CRP)

Answer 149

In the first 4-16 weeks of life Usually males (5:1 ratio) FH is common

Answer 150

Non-bilous vomiting - 'projectile' Weight loss Cap gas (alkalosis, hypochloraemia, hypokalaemia)

Answer 151

Test feed IV fluid (0. 45 N Saline/ 5% Dextrose + KCl 0. 9% Saline for NG loss) US Periumbilical pyloromyotomy

Answer 152

Malrotation and volvulus

Answer 153

Upper GI contrast study ASAP

Answer 154

Laparotomy ASAP

Answer 155

Intussusception

Answer 156

USS abdomen looking for a target sign - This is usually when the terminal ileum goes through the ileocecal valve. Bits of odematous bowel inside each other.

Answer 157

pneumostatic reduction (air enema) laparotomy

Answer 158

LBW Trisomy 21 Hypothyroidism Mucopolysaccharidosis

Answer 159

Normally self-resolves by the age of 4 Repair if there are compications Peristance over 4 years, large defect, aesthetic

Answer 160

When all of the abdomen is born outside the body - abdominal wall defect. Gut is eviscerated and exposed

Answer 161

Exomphalos

Answer 162

associated anomalies 25% cardiac 25% chromosomal - Trisomy 13, 18, 21 15% renal, neurological Beckwith-Weideman syndrome

Answer 163

Primary/delayed closure

Answer 164

Boys (2% of boys get them) Boys are 9 times more likely to get a hernia than a girl

Answer 165

Prematurity

Answer 166

Incarceration - risk is especially high in children less than 1 year of age

Answer 167

management \< 1 year URGENT referral repair - no place for observation \> 1 year elective referral and repair incarcerated reduce and repair on same admission

Answer 168

Scrotal swelling Very common in newborns Painless Increase with crying, straining, evening Bluish colour Chinese lantern effect

Answer 169

testis that cannot be manipulates into the bottom half of the scrotum

Answer 170

true cryptorchidism retractile ectopic (ascending testis) Retractile = you can pull it down but it can also jump back up inside. Ectopic – can be somewhere in the canal (superficial femoral pouch or somewhere on the other side) Ascending – as they grow the cord doesn’t increase at the same rate – usualy normal but then 5 years later they are ascended.

Answer 171

**fertility** 1% loss germs cells / month undescent…… **malignancy** * RR 3 x (probably intra-abdominal only) * lifetime risk - \<1% **trauma** (increased likelihood of rupture of they are not within the scrotum) **torsion** **cosmetic**

Answer 172

Absolute: Balanitis Xerotica Obliterans Relative: * Balanitis prosthitis * Religious * UTI (circumcision for UTI prevention is only really effective if there is an abnormal urinary tract that is predisposing the person to recurrent urinary tract infections

Answer 173

Painful Bleding Meatal stenosis Fistula Cosmetic (looks different)

Answer 174

Torsion of the testis (you have 6-8 hours to recover the testis from the onset of symptoms- if in doubt explore) Torsion appendix testis Epididymitits

Answer 175

Prevent renal scarring Reflux nephropathy and chronic renal failure, prevent hypertension

Answer 176

xall \<6/12, atypical, recurrent

Answer 177

pure growth bacteria \> 105 pyuria systemic upset fever, vomiting

Answer 178

US (looking at the size, position, shape and for hydronephrosis) Renography: * MAG3 - drainage, function, reflux * DMSA - function, scarring Micturating cystourethrogram (MCUG) (this is good for showing reflux but it is a really uncomfortable test for a child

Answer 179

conservative * voiding advice, constipation (you want to avoid constiaption), fluids antibiotic prophylaxis * ? until age 4 * Trimethoprim (2mg/kg nocte) STING (submucosal teflon injection) * mild/moderate with symptoms ureteric reimplantation

Answer 180

Upper tract Intersex

Answer 181

Do not circumsize One stage or 2 stage procedure

Answer 182

Motor Language Cognitive Social Emotional Significant delay of skills is when they are 2SD from population

Answer 183

when 2 or more domains are affected

Answer 184

A learning disability is a significant impairment in intellectual functioning and affects the person’s ability to learn and problem-solve in their daily life.It has nearly always been present since childhood.

Answer 185

ASQ (ages and stages questionnaire) PEDS (Parents evaluation of developmental status) M-CHAT (Checklist for autism in toddlers) SOGS-2 (Schedule of Growing Skills)

Answer 186

All areas of development are age appropriate Delay: Global or isolated Disorder: Abnormal progression and presentation eg Autism Regression: loss of milestones

Answer 187

Broad forehead Medial eyebrow flare Strabismus Flat nasal bridge Malar flattening Short nose, long philtrum Full lips Wide mouth

Answer 188

Learning Guide developmental red flags: - Social smile by two months - Sitting unsupported at 9 months - Standing unsupported at 18 months - No words by 2 years Positive: Loss of developmental skills Concerns re vision Concerns re hearing Floppiness No speech by 18-24 months Asymmetry of movement Persistent toe walking Head circumference \>99.6th C or \< 0.4th C Negative: Sit unsupported by 12 months Walk by 18months (boys) or 2 years (girls): Check creatinine kinase Walk other than on tiptoes Run by 2.5 years Hold objects in hand by 5 months Reach for objects by 6 months Points to objects to share interest by 2 years

Answer 189

Based on clinical abnormalities Diagnostic yield of specific tests Timing Genetic testing: chromosomal analysis, Fragile X, FISH, array CGH **Creatine kinase** **Thyroid screening** **Metabolic testing**: amino and organic acids,NH4,Lactate. **Ophthalmological examination** **Audiology assessment** Consider congenital infection **Neuroimaging**

Answer 190

Delayed maturation Cerebral palsy Developmental coordination disorder

Answer 191

abnormal tone early infancy delayed motor milestones abnormal gait feeding difficulties

Answer 192

deafness visual impairment Multisensory impairment

Answer 193

Specific Language Impairment Learning Disability

Answer 194

Autism Asperger syndrome Elective mutism

Answer 195

The family gains understanding of the condition, including prognostic information Lessens parental blame Ameliorates or prevents co-morbidity by identifying factors likely to cause secondary disability that are potentially preventable Appropriate genetic counselling Accessing more support Address concerns about possible causes e.g. events during pregnancy or delivery Potential treatment for a few conditions

Answer 196

Developmental paediatrician Speech and Language therapist OT/ PT: functional impairments and strengths Psychologist Social worker Geneticist

Answer 197

A child or young person is said to have 'additional support needs' if they need additional support with their education. Additional support can mean any kind of educational provision that is more than, or very different from, the education that is normally provided in mainstream schools

Answer 198

Community paediatrics clinics Child development teams Multidisciplinary assessment Therapy services

Answer 199

Difficulties with mainstream approaches to learning Disability or health needs, such as motor or sensory impairment, learning difficulties or autistic spectrum disorder. Family circumstances e.g. young people who are carers or parents.

Answer 200

Personal learning planning (PLP) is a way of thinking about, talking about and planning what and how a child learns. It’s also a way of assessing their progress and acting on the results of that assessment. So this means deciding how a child learns and evaluating on the go.

Answer 201

lIEP is a detailed plan for a child’s learning. It contains some specific, short-term learning targets for the child and will set out how those targets will be reached. Targets are: l Specific ● Measurable ● Achievable ● Relevant ● Timed. In some areas these plans are called additional support plans or individual support plans. Not legal documents. This is essentially just SMART goals

Answer 202

CSP - coordinated support plan. A CSP is a detailed plan of how child’s support will be provided. It is a legal document and aims to ensure all the professionals who are helping the child, work together. It also helps ensure that everyone, including parents and the child, is fully involved in that support. For children in local authority school education and needing significant additional support. Complex or multiple needs Needs likely to continue \> 1 year Support required by \> 1 agency. The parents’ guide to additional support for learning, Enquire (2016)

Answer 203

If they need extra support Access to mental health services Access to respite care This is part of the children and young people act Part of the GIFREC approach (getting it right for every child)

Answer 204

Why they need support What type of support they need How long they need support for Who should provide the support May include IEP or a CSP

Answer 205

Increase area : volume ration Deceased water content Decrease metabolic reserves

Answer 206

Ketotic hypoglycaemia

Answer 207

BP increase RR increase Lower BP

Answer 208

Baby immune system is not fully developed From 4 months to 5 years - lots of recurrent infections (before 4 months you are leaching off the immunoglobulins from the mother

Answer 209

Requires both humeral and cellular immunity

Answer 210

* Abdominal migraine * Bronchiolitis * Bronchopulmonary dysplasia * Croup * Enuresis * Febrile convulsion * Glue ear * Intraventricular haemorrhage * Necrotising enterocolitis * Non accidental injury * Sudden unexplained death of infants * Toddler’s diarrhoea * Vesico-ureteric reflux * Viral induced wheeze

Answer 211

* Asthma (COPD) * Autism * Cerebral palsy * Cystic fibrosis * Gastroschisis * Hirschsprungs disease * Spina bifida

Answer 212

Meningitis

Answer 213

* Opportunistic approach and observation skills * Appearance * Gait * Head size * Skin findings * Real world examination (depends on age) * Synthesis of history and clinical findings into a differential diagnosis and investigation plan Observation is really important before you even do any 'examination'

Answer 214

Isolated acture Chronic progressive Chronic non-progressive is likely to be tension type

Answer 215

* Growth parameters, OFC, BP (Craniopharyngioma may affect growth) * Sinuses, teeth, visual acuity * Fundoscopy (papilloedema) * Visual fields (craniopharyngioma) * Cranial bruit * Focal neurological signs * Cognitive and emotional status * The diagnosis of headache etiology is clinical

Answer 216

* Associated abdominal pain, nausea, vomiting * Focal symptoms/ signs before, during, after attack: Visual disturbance, paresthesia, weakness * ‘Pallor’ * Aggravated by bright light/ noise * Relation to fatigue/ stress * Helped by sleep/ rest/ dark, quiet room * Family history often positive

Answer 217

* Hemicranial pain * Throbbing/ pulsatile * Abdo pain, nausea, vomiting * Relieved by rest * Photophobia/ phonophobia * Visual, sensory, motor aura * Positive family history Tension: * Diffuse, symmetrical * Band-like distribution * Present most of the time (but there may be symptom free periods) * “Constant ache”

Answer 218

* Aggravated by activities that raise ICP eg. Coughing, straining at stool, bending * Woken from sleep with headache

Answer 219

* Headache is back before allowed to use another dose * Paracetamol/ NSAIDs * Particular problem with compound analgesics eg. Cocodamol

Answer 220

* Features of cerebellar dysfunction * Features of raised intracranial pressure * New focal neurological deficit eg. new squint * Seizures, esp focal * Personality change * Unexplained deterioration of school work

Answer 221

* Acute attack: effective pian relief, triptans * Preventative (at least 1/week): Pizotifen, Propranolol, Amitryptyline, Topiramate, Valproate

Answer 222

* Aim at reassurance: no sinister cause * Multidisciplinary management * Attention to underlying chronic physical, psychological or emotional problems * Acute attacks: simple analgesia * Prevention: Amitryptiline * Discourage analgesics in chronic TTH

Answer 223

•An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons

Answer 224

* Epilepsy: A tendency to recurrent, unprovoked (spontaneous) epileptic seizures * A question that must be answered clinically, with recourse to EEG only for supportive evidence * A seizure is not necessarily epileptic * Consequences of misdiagnosis of epilepsy can be serious

Answer 225

* Acute symptomatic seizures: due to acute insults eg. Hypoxia-ischaemia, hypoglycemia, infection, trauma * Reflex anoxic seizure: common in toddlers * Syncope * Parasomnias eg. night terrors * Behavioural stereotypies (this can include rocking and head banging) * Psychogenic seizures (NEAD) (non epileptic attack disorder)

Answer 226

Febrile convulsion

Answer 227

•An event occurring in infancy/ childhood, usually between 3 months and 5 years of age, associated with fever but without evidence of intracranial infection or defined cause for the seizure

Answer 228

* Distinguishing seizure types can be challenging * Jerk/ shake: clonic, myoclonic, spasms * Stiff: usually a tonic seizure * Fall: Atonic/ tonic/ myoclonic * Vacant attack: absence, complex partial seizure

Answer 229

- Decreased inhibition (gama-amino-butyric acid, GABA) - Excessive excitation (glutamate and aspartate) - Excessive influx of Na and Ca ions

Answer 230

Both hemispheres are involved in a generalised seizure

Answer 231

30-60% - this is because you might not pick up any epileptic discharge - you can also get false positives •Useful in identifying seizure types, seizure syndrome and etiology

Answer 232

arryythmia may be causing hypoxia which caused the seizure

Answer 233

* History * Video recording of event * ECG in convulsive seizures * Interictal/ ictal EEG * MRI Brain: to determine etiology eg. Brain malformations/ brain damage * Genetics: idiopathic epilepsies are mostly familial; also single gene disorders eg. Tuberous sclerosis * Metabolic tests: esp if associated with developmental delay/ regression

Answer 234

* Anti-epileptic drugs (AED) should only be considered if diagnosis is clear even if this means delaying treatment * Role of AED is to control seizures, not cure the epilepsy * Start with one AED: slow upward titration until side-effects manifest or drug is considered to be inefficient. * Age, gender, type of seizures and epilepsy should be considered in selecting AEDs * S/Es: CNS related can be detrimental; Drowsiness, effect on learning, cognition and behavioural * Sodium Valproate: first line for generalised epilepsies (not in girls- recent MHRA advice) * Carbamazepine: first line for focal epilepsies * Several new AEDs with more tolerability and fewer side effects: Levatiracetam, Lamotrigine, Perampanel * Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for resistant epilepsies) Vagal nerve stimulator - implantable device that releases electrical signals into the vagus nerve - breaks the epileptic activity i nthe seizure - it is something you can activate when there is a seizure actually happening. Epilepsey surgery (mesial temporal lobe epilepsy - surgery can be curative)

Answer 235

* Baby ‘floppy’ from birth * Slips from hands * Paucity of limb movements * Alert, but less motor activity * Delayed motor milestones * Able to walk but frequent falls

Answer 236

Gowers sign

Answer 237

Pescavus High foot arch

Answer 238

Myaesthenic syndromes

Answer 239

Spinal muscular atrophy (motor neurones)

Answer 240

Xp21, dystrophin gene

Answer 241

•Symmetrical proximal weakness Waddling gait, calf hypertrophy Gower’s sign positive Cardiomyopathy Respiratory involvement in teens Steroids are the mainstay of treatment

Answer 242

Creatinine kinase

Answer 243

Suicide Non-intentional injuries and poisoning Road traffic accident

Answer 244

* Highest graft failure rates * ~35% lose kidney * 1.8 times the rate of people \<17 and \>24 years * Substantial costs to individuals & NHS * Deterioration in HbA1c in diabetes * Associated with lasting complications (e.g., cardiovascular disease, neuropathjy, retinopathy). * Consistent across medical conditions This is because: * Different priorities * Different thought processes/ability to process long term outcomes

Answer 245

* HEADSS * Home * Education (or Employment) * Activities * Drugs/Alcohol * Sexuality * Suicide/Self Harm

Answer 246

Leukaemias (most common) Braintumours

Answer 247

* Down * Fanconi * BWS * Li-Fraumeni Familial Cancer Syndrome * Neurofibromatosis

Answer 248

Radiation Infection (ebstein barr virus can lead to lymphoma, Papilloma virus)

Answer 249

´**Acute** * Hair loss * Nausea & vomiting * Mucositis * Diarrhoea / constipation * Bone marrow suppression – anaemia, bleeding, infection ´**Chronic** * Organ impairment – kidneys, heart, nerves, ears * Reduced fertility * Second cancer

Answer 250

´**Acute** * Lethargy * Skin irritation * Swelling * Organ inflammation – bowel, lungs ´**Chronic** * Fibrosis / scarring * Second cancer * Reduced fertility

Answer 251

´Risks ´ANC \< 0.5 x 109 ´Indwelling catheter ´Mucosal inflammation ´High dose chemo / SCT Typs of infection : ´Pseudomonas aeruginosa ´Enterobacteriaciae eg E coli, Klebsiella ´Streptococcus pneumoniae ´Enterococci ´Staphylococcus ´Fungi eg. Candida, Aspergillus

Answer 252

´Fever (or low temp) ´Rigors ´Drowsiness ´Shock ´Tachycardia, tachypnoea, hypotension, prolonged capillary refill time, reduced UO, metabolic acidosis

Answer 253

IV access Blood culture, FBC, coag, UE, LFTs, CRP, lactact CXR Other ◦Urine microscopy / culture ◦Throat swab ◦Sputum culture / BAL ◦LP ◦Viral PCRs ◦CT / USS ABC ◦Oxygen ◦Fluids Broad spectrum antibiotics Inotropes PICU

Answer 254

Early * early morning headache/vomiting * tense fontanelle * increasing HC (hydrcephalus) Late * constant headache * papilloedema * diplopia (VI palsy) * Loss of upgaze * neck stiffness * status epilepticus, * reduced GCS * Cushings triad (low HR, high BP)

Answer 255

* Imaging is mandatory (if safe) * CT is good for screening * MRI is best for more accurate diagnosis

Answer 256

* Dexamethasone if due to tumour * Reduce oedema and increase CSF flow * 250 micro/kg IV STAT then 125 microg/kg BD * Neurosurgery - urgent CSF diversion * Ventriculostomy – hole in membrane at base of 3rd ventricle with endoscope * EVD (temporary) * VP shunt

Answer 257

* Potential complication of nearly all paediatric malignancies * Affects 5 % of all children with cancer * 10-20 % Ewing’s or Medulloblastoma * 5-10 % Neuroblastoma & Germ cell tumour * Diagnosis (65 %), relapse, progression * Pathological processes * Invasion from paravertebral disease via intervertebral foramina (40 % extradural) * Vertebral body compression (30 %) * CSF seeding (20 % intradural, extraspinal) * Direct invasion (10 % intraspinal)

Answer 258

* Symptoms vary with level * weakness (90 %) * pain (55-95 %) * sensory (10-55%) * sphincter disturbance (10-35%)

Answer 259

´Urgent MRI ´Start dexamethasone urgently to reduce peri-tumour oedema ´Definitive treatment with chemotherapy is appropriate when rapid response is expected ´Surgery or radiotherapy are other options ´ ´Outcome depends on severity of impairment rather than duration between symptoms and diagnosis ´Mild impairment \> 90 % recovery ´Paraplegic 65 % recovery

Answer 260

* Lymphoma * Other – neuroblastoma, germ cell tumour, thrombosis

Answer 261

* SVCS: facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS * SMS: dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea Plethora = large or excessive amount of swelling

Answer 262

* Investigation * CXR / CT chest (if able to tolerate) * Echo

Answer 263

* Keep upright & calm * Urgent biopsy (ideally) * Look to obtain important diagnostic information without GA * FBC, BM, pleural aspirate, GCT markers * Definitive treatment is required urgently * Chemotherapy is usually rapidly effective * Presumptive treatment may be needed in the absence of a definitive histological diagnosis (steroids) * Radiotherapy is effective * May cause initial increased respiratory distress * Rarely surgery if insensitive * CVAD-associated thrombosis should be treated by thrombolytic therapy * Most of underlying malignancies have a good prognosis

Answer 264

* Metabolic derangement * Rapid death of Tumour Cells * Release of intracellular contents * At or shortly after presentation * Secondary to treatment * (rarely spontaneous)

Answer 265

* increase potassium * increase urate, relatively insoluble * increase phosphate * decrease calcium * Acute renal failure * Urate load * CaPO4 deposition in renal tubules

Answer 266

* Avoidance * ECG Monitoring * Hyperhydrate-2.5l/m2 * QDS electrolytes * Diuresis * Never give potassium * ¯ uric acid * Urate Oxidase-uricozyme (rasburicase) * Allopurinol * Treat hyperkalaemia * Ca Resonium * Salbutamol * Insulin * Renal replacement therapy

Answer 267

0-3months 200g 3-6 months 150g 6-9 months 100g 9-12 months 75-50g Doubl weight by 6 months and triple by one year After 1 year approximately 2 kg and 5 cm per year until puberty

Answer 268

Nutritionally best for full term babies Well tolerated Less allergenic Low renal solute load Ca:PO4, LCP FAs Improves cognitive development Reduces infection Macrophages and lymphocytes Interferon, lactoferrin, lysozyme Bifidus factor

Answer 269

* Have a written breast-feeding policy- routinely communicated to all staff. * Train all health care staff in skills necessary to implement this policy. * Inform all pregnant women about the benefits/management of breastfeeding. * Help mothers initiate breastfeeding within a half-hour of birth. * Show mothers how to breastfeed, and how to maintain lactation even if they should be separated from their infants. * Give newborn infants no food and drink other than breast milk, unless medically indicated. * Practise rooming-in - allow mothers+ infants to remain together - 24h/day * Encourage breast-feeding on demand. * Give no teats or dummies to breastfeeding infants. * Foster the establishment of breast-feeding support groups and refer mothers to them on discharge from the hospital or clinic.

Answer 270

Milk is the exclusive feed for 4-6 months

Answer 271

1 in 20 under 2’s will react to a food Cows’ milk most commonly. Majority are delayed, non IgE reactions eg vomiting, diarrhoea, abdominal discomfort/ distension,eczema Only test is trial of CMP exclusion (skin testing has to be IgE mediated)

Answer 272

4 week trial of milk avoidance Special formula or milk free diet for breast feeding mums Reintroduction at 4 weeks unless clear benefit Re challenge after 6 months of improvement Milk ladder approach Not all forms of milk equally allergenic Helps achieve earlier tolerance 60-70% on normal diet by 1 year

Answer 273

Extensively hydrolysed protein feeds Second line feeds are amino acid based feeds - (babies with severe collitis, enteropathy / symptoms on breast milk)

Answer 274

Short lived condition eg post gastro enteritis Confused with cows milk protein intolerance Lactose free milks are not CMP free

Answer 275

Milk allergy when hydrolysed formulae refused Vegan families, if not breast fed Consider for children\>1 year still on milk free diet

Answer 276

Why wean? Milk alone is inadequate Source of vitamins and trace elements Man is an omnivore Encourage tongue and jaw movements in preparation for speech and social interaction

Answer 277

Vitamin D (we still see rickets) Dark skinned children not on vitamin drops at risk Prolonged breast feeding and mum not on Vit D

Answer 278

**•Pre-ejection phase** * Pallor * Nausea * Tachycardia **•Ejection Phase** * Retch * Vomit **•Post-ejection Phase**

Answer 279

* Enteric pathogens * Intestinal inflammation * Metabolic derangement * Infection * Head injury * Visual stimuli * Middle ear stimuli

Answer 280

Metabolic alkalosis? High pH Low potassium Low chlorine

Answer 281

Gastroesophageal reflux Overfeeding Pyloric stenosis

Answer 282

* Babies 4-12 weeks * Boys \> Girls * Projectile non-bilious vomiting * Weight loss * Dehydration +/- shock * Characteristic electrolyte disturbance: –Metabolic alkalosis (↑pH) –Hypochloraemia (↓Cl) –Hypokalaemia (↓K)

Answer 283

* Should always ring alarm bells * Due to intestinal obstruction until proved otherwise * Causes Intestinal atresia (in newborn babies only) Malrotation +/- volvulus Intussusception Ileus Crohn’s disease with strictures

Answer 284

Abdominal x-ray Consider contrast meal Surgical opinion re exploratory laparotomy

Answer 285

* This is almost always due to **gastro-oesophageal reflux** * Very common problem in infants * Self limiting and resolves spontaneously in the vast majority of cases * A few exceptions: **–Cerebral palsy** **–Progressive neurological problems** **–Oesophageal atresia +/- TOF operated** **–Generalised GI motility problem**

Answer 286

•Gastrointestinal –Vomiting –Haematemesis •Nutritional –Feeding problems –Failure to thrive •Respiratory –Apnoea –Cough –Wheeze –Chest infections •Neurological –Sandifer’s syndrome Sandifer's syndrome is the association of gastro-oesophageal reflux disease with spastic torticollis and dystonic body movements. Nodding and rotation of the head, neck extension, gurgling sounds, writhing movements of the limbs, and severe hypotonia have been reported. It is hypothesised that such positionings provide relief from discomfort caused by acid reflux. A causal relation between gastro-oesophageal reflux disease and the neurological manifestations of Sandifer's syndrome is supported by the resolution of the manifestations on successful treatment of gastro-oesophageal reflux disease. The clinical manifestations almost invariably arouse the suspicion of neurological disease and lead to unnecessary investigative procedures.

Answer 287

* History & examination often sufficient * Radiological investigations –Video fluoroscopy –Barium swallow * pH study * Oesophageal impedance monitoring * Endoscopy

Answer 288

•Barium swallow –Aims: * Dysmotility * Hiatus hernia * Reflux * Gastric emptying * strictures –Problems: •Aspiration Inadequate contrast taken (NG tube)

Answer 289

* Thickeners for liquids * Appropriateness of foods –Texture –Amount •Behavioural programme –Oral stimulation –Removal of aversive stimuli •Feeding position

Answer 290

* Calorie supplements * Exclusion diet (milk free) * Nasogastric tube * Gastrostomy

Answer 291

•Feed thickener Gaviscon Thick & Easy * Prokinetic drugs * Acid suppressing drugs H2 receptor blockers Proton pump inhibitors

Answer 292

•Failure of medical treatment –Persistent: * Failure to thrive * Aspiration * Oesophagitis

Answer 293

Nissen fundoplication

Answer 294

•Children with cerebral palsy are more likely to have complications of bloat, dumping and retching after surgery Dumping syndrome - rapid gastric emptying leading to unpleasant symptoms such as intermittent sweating and diarrhoea following meals

Answer 295

–4 or more stools per day –For more than 4 weeks –\<1 week: acute diarrhoea –2 to 4 weeks: persistent diarrhoea –\>4 weeks: chronic diarrhoea

Answer 296

**•Motility disturbance** * Toddler Diarrhoea * Irritable Bowel Syndrome **•Active secretion (secretory)** * Acute Infective Diarrhoea\* * Inflammatory Bowel Disease **•Malabsorption of nutrients (osmotic)** * Food Allergy\* * Coeliac Disease\* * Cystic Fibrosis

Answer 297

Movement of water into the bowel to equilibrate osmotic gradient Usually a feature of malabsorption (enzymatic defect, transport defect) Mechanism of action of lactulose / movicol

Answer 298

Toxin production from Vibrio cholerae and enterotoxigenic escherichiea coli Intestinal fluid secretion predominantly driven by active Cl- secretion via CFTR

Answer 299

Toddler's diarrhoea Irritable bowel syndrome Congenital hyperthyroidism Chronic intestinal pseudo-obstruction

Answer 300

secretory effect of cytokines Accelerated transit time in response to inflammation Protein exudate across inflamed epithelium

Answer 301

Lack of lipase and resultant steatorrhoea Classically cystic fibrosis

Answer 302

* Abdominal bloatedness * Diarrhoea * Failure to thrive * Short stature * Constipation * Tiredness * Dermatitis herpatiformis

Answer 303

•Serological Screens –Anti-tissue transglutaminase –Anti-endomysial –Anti-gliadin (not really tested anymore) –Concurrent IgA deficiency in 2% may result in false negatives * Gold standard- duodenal biopsy (lymphocytic infiltration of surface epithelium, partial / total villous atrophy, crypt hyperplasia * Genetic testing - HLA DQ2, DQ8

Answer 304

* Symptomatic children * Anti TTG \>10 times upper limit of normal * Positive anti endomysial antibodies * HLA DQ2, DQ8 positive

Answer 305

* Gluten-free diet for life * Gluten must not be removed prior to diagnosis as serological and histological features will resolve * In very young \<2yrs, re-challenge and re-biopsy may be warranted * Increased risk of rare small bowel lymphoma in untreated

Answer 306

* Pneumonia, bronchiolitis * Meningitis * Septicaemia Rhinitis is more common in winter months

Answer 307

usually no longer than 14 days

Answer 308

Drum no longer transparent and shiny Eardrum is being pushed forward by pus – about to burst

Answer 309

Normally lasts less than 7 days

Answer 310

Usualy self-limiting - secondary infection with pneumococcus / H'flu The primary infection is viral Can cause spontaneous rupture of the eardrum (antibiotic treatment does not usuall help, causes side effects and condition is usually getting better by the time antibiotics usually start working)

Answer 311

oxidation nutrition hydration Analgesia

Answer 312

oxygenation, hydration are rubbish in epiglottitis because they are usually septic

Answer 313

•Bacterial overgrowth –Strep pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Mycoplasma pneumoniae, Chlamydia pneumoniae •Viral infection –RSV, parainfluenza III, influenza A and B, adenovirus, rhinovirus

Answer 314

* Common ++++ * Loose rattly cough * Post-tussive vomit - “glut” * Chest free of wheeze/creps Child is very well but the parent is very worried Usually in children between 6 months and 4 years Goes on for a very long time

Answer 315

Haemophilus / pneumococcus

Answer 316

•Disturbed mucociliary clearance –Minor airway malacia –RSV/adenovirus * Lack of social inhibition! * Bacterial overgrowth is secondary

Answer 317

* Age \<6 mo, \>4yr * Static weight * Disrupts child’s life * Associated SOB (when not coughing) * Acute admission * Other co-morbidities (neuro/gastro)

Answer 318

Usually between 7 days and 25 days

Answer 319

•Usually RSV, others include paraflu III, HMPV

Answer 320

* Nasal stuffiness, tachypnoea, poor feeding * Crackles +/- wheeze

Answer 321

Christmas time (week 51)

Answer 322

Less than 12 months of age One off disease - not recurrent Follows typical history

Answer 323

NPA Oxygen saturations No routine for: - CXR - Bloods - Bacterial cultures

Answer 324

* 48 hrs, fever (\>38.5oC), SOB, cough, grunting * Wheeze makes bacterial cause unlikely * Reduced or bronchial breath sounds * “Infective agents” –Virus+commensal bacteria/bacterium

Answer 325

•You might call it pneumonia if –Signs are focal, ie in one area (LLZ) –Creps –High fever Try and avoid calling it pneumonia because it causes great anxiety in the parent

Answer 326

•Investigations –CXR and inflammatory makers NOT “routine”

Answer 327

•Management –Nothing if symptoms are mild –(always offer to review if things get worse!) –Oral Amoxycillin first line –Oral Macrolide second choice –Only for iv if vomiting

Answer 328

Non-severe LRTI When child is not vomitting Oral antibiotics are assocaited with a shorter hospital stay, they are cheaper but they ahve a fever for a few more hours.

Answer 329

* Literally “panting” * Chronic * Wheeze, cough and SOB * Multiple triggers * URTI, exercise, allergen, cold weather, etc * Variable/reversible * Responds to asthma Rx * No uniform definition Key words: - Wheee - Variability - Responds to treatment

Answer 330

Genes Inherently abnormal lungs Early onset atopy Later exposures: Rhinovirus Exercise Smoking

Answer 331

* All in the history! * Examination unhelpful –Unlikely to be wheezing –Stethoscope never important (often unhelpful) •No asthma test in children –Peak flow random number generator –Allergy tests irrelevant –Spirometry lacks specificity –Exhaled nitric oxide unproven

Answer 332

* Everyone coughs! * Dry * Nocturnal (just after falling asleep) * Exertional

Answer 333

Wheeze (with and without URTI) SOB@rest Parental asthma Responds to treatment (2 month trial of ICS, you can confirm by giving them a medicaion holiday as well) •Simplistically –Under 18 months, most likely infection –Over 5 years, most likely asthma –BUT if it sounds like asthma and responds to asthma it is asthma regardless of age!

Answer 334

Onset under 5 years: ## Footnote * Congenital * CF * PCD * Bronchitis * Foreign body Onset over 5 years: * Dysfunctional breathing * Vocal cord dysfunction * Habitual cough * Pertussis

Answer 335

The goals of treatment are: * “minimal” symptoms during day and night * minimal need for reliever medication * no attacks (exacerbations) * no limitation of physical activity * normal lung function (in practical terms FEV1 and/or PEF \>80% predicted or best)

Answer 336

* Closed questions * SANE * Short acting beta agonist/week * Absence school/nursery * Nocturnal symptoms/week * Excertional symptoms/week

Answer 337

•If not well controlled –Not taking treatment –Not taking treatment correctly –Not asthma (Stop asthma Rx) –None of the above Increase Rx

Answer 338

* Max dose ICS 800 microg (\<12 yo) * No oral B2 tablet * LTRA first line preventer in \<5s * No LAMAs * Only two biologicals

Answer 339

When? * Diagnostic test * B2 agonists \>two days a week * symptomatic three times a week or more, or waking one night a week. * exacerbations of asthma in the last two years What with? •Start very low dose inhaled corticosteroids (or LTRA in \<5s)

Answer 340

Height suppression Oral candidiasis? Adrenocortical suppression

Answer 341

Initial add on preventer ## Footnote Gets complicated * Add on LABA or LTRA (BTS/SIGN) * Add on LTRA (NICE) * Increase ICS dose (GINA) LABA have to be used with an ICS - used as a fixed dose inhaler

Answer 342

Montelukast

Answer 343

* Add on LABA but keep an open mind! * Additional add-on therapies –Increase ICS –LTRA

Answer 344

At the beginning of the fourth week

Answer 345

Protects lungs against circulatory overload Allows the right ventricle to strengthen Carries low oxygen saturated blood

Answer 346

Connects the umbilical bein to the IVC | (carries mostly oxygenated blood)

Answer 347

Radiation: Heat dissipated to colder objects. Convection: Heat loss by moving air. Evaporation: We are born in the water. Conduction: Heat loss to surface on which baby lies

Answer 348

Non invasive: Blood gas determination PaCO2 5-6 kPa, PaO2 8-12 kPa Trans-cutaneous pCO2/O2 measurement

Answer 349

Invasive: Capnography Tidal volume 4-6 ml/kg Minute ventilation: Tidal Volume ml/kg x respiratory rate Flow-volume loop.

Answer 350

Appears on Day of life (DOL) 2-3. Disappears within 7-10 DOL in term infants and up to 21 DOL in premature infants

Answer 351

Shift of interstitial fluid to intravascular Diuresis (it is normal not to pass urine for the first 24 hours) Increased loss through kidney (slower GFR, reduced Na reabsorption, decreased ability to concentrate or dilute urine), increased insensible water loss

Answer 352

15-20 g/l Week 10 = 11.4 g/l

Answer 353

Reduced erythropoesis Infection Blood letting - most important cause

Answer 354

less than 10th centile for growth?

Answer 355

Smoking Maternal pre-eclamptic toxemia Chromosomal - Edwards syndrome Infection - CMV Placental abruption Twin pregnancy

Answer 356

Common problems: - Perinatal Hypoxia - Hypoglycaemia - Hypothermia - Polycythaemia - Thrombocytopenia - Hypoglycaemia - Gastrointestinal problems (feeds, NEC) - RDS, Infection

Answer 357

Hypertension Reduced growth Obesity Ischaemic heart disease

Answer 358

less than 28 weeks

Answer 359

less than 2500 grams

Answer 360

Less than 1500 grams

Answer 361

Less than 100 grams

Answer 362

5-12 percent

Answer 363

Antenatal steroids

Answer 364

Surfactant Early intubation Non-invasive support (N-CPAP) Minimal ventilation (low tidal volume and good inflation)

Answer 365

RDS PDA Necrotizing enterocollitis Retiopathy of prematurity Intraventricular haemorrhage

Answer 366

Lack of surfactant secreted by type 2 pneuocytes

Answer 367

Respiratory Distress Tachycardia Hypoxia Chest X-Ray (ground glass appearance)

Answer 368

Antenatal steroids Exogenous surfactant Respiratory support (invasive ventilation / continuous positive airway pressure) Complications include chronic lung disease

Answer 369

Blood shunted from left to right Fluid overload Heart failure

Answer 370

Continuous murmur heard between clavicles Bounding pulses Wide pulse pressure

Answer 371

Fluid restriction Ibuprofen/indometacin

Answer 372

Heart failure Failure to wean off respiratory support

Answer 373

Ischaemia of gut wall and secondary infection of bowel Risk fafctors: - Prematurity - Milk feeding Sepsis Ibuprofen / indometacin

Answer 374

Intolerance of feeds Distended abdomen Vomiting Rectal bleeding Abdomainl X-Ray = distended bowel loops, bowel wall thickening, intramural air, perforation

Answer 375

Nil by mouth, total parenteral nutrition, antibiotics, surgery if severe or perforation (bowel resection and stoma formation)

Answer 376

Malabsorption Stricture

Answer 377

Haemorrhage of fragil eblood vessels in germinal matrix secondary to hypoxia / RDS

Answer 378

Grading of the bleed Depends if the bleed is periventricular or spreads to the ventricles / parenchyma

Answer 379

Ventricular dilatation (CSF taps / shunt insertion)

Answer 380

Rhesus incompatibility ABO incompatibility G6PD deficiency Hereditary spherocytosis Sepsis Bruising

Answer 381

Physiological/breast milk, causes of 24 hour jaundice

Answer 382

Unconjugated: physiological/breast milk. UTI, hypothyroidism, galactosaemia Conjugated: Biliary artresia, neonatal hepatitis

Answer 383

SIRS = systemic inflammatory response syndrome * Fever or hypothermia * Tachycardia * Tachypnoea • Leucocytosis or leucocytopaenia Infection = bacteraemia (e.g. bacteria multiplying in the bloodstream)

Answer 384

Sepsis and multi-organ failure: 2 or more of the following: • Respiratory failure * Renal failure * Neurologic failure * Haematological Failure • Liver failure ARDS (inflammatory response in the lungs) Septic shocke

Answer 385

Neutrophils and monocytes (there is also activation of compliment)

Answer 386

Fever or hypothermia Cold hands/feet, mottled Prolonged capillary refill time Chills/rigors Limb pain Vomiting and/or diarrhoea Muscles weakness Muscle/joint aches Skin rash Diminished urine output

Answer 387

Nuchal rigidity Headaches, Photophobia Diminished consciousness Focal neurological abnormalities Seizures In neonates: Lethargy, Irritability Bulging fontanelle ‘nappy pain’

Answer 388

Temperature below 36 or above 38 Innappropriate tachycardia Poor peripheral perfusion/ capillary refill greater than 2 secs / mottled Altered mental state Innapropriate tachypnoea Hypotension

Answer 389

Supportive treatment: A airway B breathing C circulation DEFG = ‘don’t ever forget glucose’ Causative treatment: Antibiotics with good penetration in CSF & broad-spectrum: 3rd generation cefalosporins (+ amoxicilline if neonate) • Chemoprophylaxis Close household contacts Meningococcus B and Streptococcus group A

Answer 390

Blood: FBC; leucocytosis, thrombocytopaenia CRP; elevated coagulation factors; low levels due to DIC blood gas; metabolic acidosis glucose; hypoglycaemia CSF: pleocytosis, increased protein level, low glucose Blood and CSF cultures (antigen testing, PCR) Urine culture, skin biopsy culture (this is to diagnose meningococcal infection). Imaging: CT-cerebrum

Answer 391

Encapsulated H. influenzae, 6 serotypes • Resist phagocytosis and complement-mediated lysis Unencapsulated H. influenzae = non-typeable H. influenzae (NTHI)

Answer 392

Bacteraemia Meningitis (as severe as pneumococcal meningitis), pneumonia, epiglottitis

Answer 393

Casues septic shock and meningitis IT is found in the nasopharynx

Answer 394

Lots of release of lipopolysaccharide (endotoxin)

Answer 395

The vaccine is recommended for babies aged eight weeks, followed by a second dose at 16 weeks, and a booster at one year.

Answer 396

The meningitis C vaccine offers protection against a type of bacteria - meningococcal group C bacteria - that can cause meningitis. Babies are offered a combined Hib/Men C vaccine at one year of age.

Answer 397

The meningitis ACWY vaccines offers protection against four types of bacteria that can cause meningitis – meningococcal groups A, C, W and Y. Young teenagers, sixth formers and "fresher" students going to university for the first time are advised to have the vaccination.

Answer 398

Arthritis for at least 6 weeks Morning stiffness or gelling irritability or refusal to walk in toddlers School absence or limited ability to participate in physical activity Rash /fever Fatigue Poor appetite/wt loss Delayed puberty

Answer 399

Septic arthritis Transient synovitis (very common in winter, runny nose, on day 3 maybe have a limp - should settle in 3/4 days, synovail inflammation secondary to an infection, self-resolves. Malignancies i.e lymphoma, neuroblastoma, bone tumours Recurrent haemarthrosis (haemophilia) Vascular abnormalities (AV malformations) Trauma others

Answer 400

Swelling:periarticular soft tissue edema/intraarticular effusion/hypertrophy of synovial membrane Tenosynovitis(swollen tendons) pain Joint held in position of maximum comfort range of motion limited at extremes.

Answer 401

Mostly boys over 8 years Test negative for ANA No extraarticular manifestation Hip onvolvement

Answer 402

Mostly girls between 1-5 years old 20-30% develop iridocyclitis Test positive for ANA Joints commonly affected: knees ankles, hands, feet and wrists No hip involvement

Answer 403

Five or more joints affected Few or no systemic manifestations (fever, slight hepatosplenomegaly, lymphadeopathy, pericarditis and chronic uveitis) Can either be seropositive (less common, classically in children over 8) or seronegative classically common in children under 5 years Temperomandibular joint in jury is common leading to limited bite and micrognathia Onset can be acute but mostly insidious Large and fast growing joints are mostly affected

Answer 404

These are 10% of all JIA patients (more common in children over 8)

Answer 405

20-25% of all JIA patients More common in children under 5

Answer 406

Usualy has 2 of the following: - Onset of polyarticular/oligoarticular arthritis in a boy over 8 years of age HLA B27 positivity Acute anterior uveitis Inflammatory spinal pain sacroiliac joint tenderness Family history of enthesitis related JIA

Answer 407

All are HLA B27 positive Family history of psoriasis Dactylitis (finger or toe inflammation) Onycholysis : nail pitting

Answer 408

Arthritis Intermittent fever for over two weeks (normal in the morning but high in the evening) Salmon red rash on the trunk and the thighs tha accompany the fever (can be brought on by scratching - koebner's phenomenon) Generalised lymphadenopathy Serositis Hepatomegaly/splenomegaly

Answer 409

NSAIDs DMARDS Bioligical agents Intra-articular oral steroids 1st line therapy is simple pain killers and NSAIDs 2nd line therapy is methotrexate, anti-TNF, Il-1 antagonist, IL-6 antagonist The role of steroids: - Used to control fever and pain - Used in severe disease complications such as pericardial effusion, tamponade, vasculitis, severe autoimmune anaemia and severe eye disease. - As a bridge between DMARDs - CHildren undergoing surgery Intra-articular steroids (mainly for oligoarticular JIA), local steroids can be used for ANA positive oligoarticular eye disease

Answer 410

Labs Plain X-Ray US MRI with contrast

Answer 411

Pharmacologic management consisting of nonsteroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs (DMARDs), biologic agents, [30] and intra-articular and oral steroids Psychosocial factors, including counselling for patients and parents School performance, such as school-life adjustments, and physical education adjustments Nutrition, particularly to address anemia and generalized osteoporosis Physical therapy to relieve pain and to address range of motion, muscle strengthening, activities of daily living, and conditioning exercises Occupational therapy, including joint protection, a program to relieve pain, range of motion, and attention to activities of daily living

Answer 412

Oligoarticular JIA

Answer 413

Failure to respond to DMARDs Anti-TNF agents are commonly used

Answer 414

Uveitis associated with JIA If untreated can progress to chronic uveitis All children diagnosed with JIA undergo screening. Early detection prevents complications

Answer 415

ANA positive oligoarticular JIA

Answer 416

Rarely symptomatic Red eyes, headache, reduced vision. Cataracts, glaucoma and blindness

Answer 417

Not detected by opthalmoscopy All JIA pts to be seen within 6 weeks of diagnosis. High risk children Initially topical steroids to reduce inflammation More severe need systemic steroids Poor response to steroids DMARD and biologics Early detection and treatment prognosis good.

Answer 418

Poor growth Localised growth disturbances Micrognathia Contractures Ocular complications

Answer 419

Strep pyogenes or staph aureus

Answer 420

Group A beta haemolytic strep - typically strep pyogenes

Answer 421

Children aged 2-6 peak incidence is age 4

Answer 422

Scarlet fever is spread via the respiratory route by inhaling or ingesting respiratory droplets or by direct contact with nose and throat discharges, (especially during sneezing and coughing).

Answer 423

fever: typically lasts 24 to 48 hours malaise, headache, nausea/vomiting sore throat 'strawberry' tongue rash - fine punctate erythema ('pinhead') which generally appears first on the torso and spares the palms and soles. Children often have a flushed appearance with circumoral pallor. The rash is often more obvious in the flexures. It is often described as having a rough 'sandpaper' texture. Desquamination occurs later in the course of the illness, particularly around the fingers and toes

Answer 424

Throat swab Antibiotic treatment should be commenced immediately before waiting for results§

Answer 425

Management ## Footnote oral penicillin V for 10 days patients who have a penicillin allergy should be given azithromycin children can return to school 24 hours after commencing antibiotics scarlet fever is a notifiable disease

Answer 426

Exotoxins of staph aureus, usually happens in kids that are less than 5 eyars of age Consists of fluid filled blisters that rupture easily, especially in the skin folds

Answer 427

Passmedicine: high-grade fever which lasts for \> 5 days. Fever is characteristically resistant to antipyretics conjunctival injection bright red, cracked lips strawberry tongue cervical lymphadenopathy red palms of the hands and the soles of the feet which later peel Fever for at elast 5 days and four of the five: **bilateral conjunctival injection** changes of the mucous membranes **cervical lymphadenopathy** polymorphous rash changes of the extremities **peripheral oedema** **peripheral erythema** **periungual desquamation**

Answer 428

It is described as a self-limited vasculitis of medium sied arteries It may cause potentially serious problems such as coronary artery aneurysms

Answer 429

Clinical diagnosis, no specific test

Answer 430

Management ## Footnote high-dose aspirin intravenous immunoglobulin echocardiogram (rather than angiography) is used as the initial screening test for coronary artery aneurysms

Answer 431

Reye's syndrome

Answer 432

Henoch-Schonlein purpura (HSP) is an IgA mediated small vessel vasculitis. There is a degree of overlap with IgA nephropathy (Berger's disease). HSP is usually seen in children following an infection. Features ## Footnote palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs abdominal pain polyarthritis features of IgA nephropathy may occur e.g. haematuria, renal failure

Answer 433

Measles Rubella Enterovirus CMV

Answer 434

Varicella zoster Herpes simplex Enterovirus

Answer 435

Rubella Enterovirus Cytomegalovirus

Answer 436

14 (10-21) days

Answer 437

Clinical features (tend to be more severe in older children/adults) ## Footnote fever initially itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular - can cause scarring systemic upset is usually mild

Answer 438

Secondary strep/staph skin infections **Meningoencephalitis**, cerebellitis, **arthritis**

Answer 439

Management is supportive ## Footnote keep cool, trim nails calamine lotion school exclusion\*: children should be kept away from school for at least 5 days from onset of rash (and not developing new lesions) immunocompromised patients and newborns with peripartum exposure should receive varicella zoster immunoglobulin (VZIG). If chickenpox develops then IV aciclovir should be considered

Answer 440

HSV 1 and HSV 2 1 = oral 2 = genital

Answer 441

primary infection: may present with a severe gingivostomatitis cold sores painful genital ulceration

Answer 442

Management gingivostomatitis: oral aciclovir, chlorhexidine mouthwash cold sores: topical aciclovir although the evidence base for this is modest genital herpes: oral aciclovir. Some patients with frequent exacerbations may benefit from longer term aciclovir

Answer 443

Enteroviruses; coxsackie A16 and enterovirus 71

Answer 444

mild systemic upset: sore throat, fever oral ulcers followed later by vesicles on the palms and soles of the feet

Answer 445

symptomatic treatment only: general advice about hydration and analgesia reassurance no link to disease in cattle children do not need to be excluded from school\*

Answer 446

Encephalitis

Answer 447

Smear of vesicle (ulcer base) - Tzanck test: no differentiation (HSV/VSV) Serology (past infections only) PCR (fluids, CSF, blood)

Answer 448

**Antibody deficiencies** - defective B cell function and therefore deficiency in one or more classes of antibodies **Cellular immunodeficiencies -** impairment of T-Cell function or the absence of normal T cells **Innate immune disorders** - defects in phagocyte function, complement deficiencies, absence or polymorphisms in pathogen recognition receptors

Answer 449

Recurrent bacterial infections of the upper and/or lower respiratory tract S.pneumoniae, H.Influenzae

Answer 450

Unusual or opportunistic infections often combined with a failure to thrive Pneumocystic jirovecii, CMV (pneumonia)

Answer 451

Defects in phagocyte function: - S.aureus (sepsis, skin lesions, abscesses internal organs) - Aspergillus infections (lung, bones and the brain) Complement deficiencies - (N.meningitidis)

Answer 452

Lack of NADPH oxidase reduces ability of phagocytes to produce reactive oxygen species

Answer 453

Recurrent pneumonias and abscess formation (particularly due to stpah aureus infection and fungi)

Answer 454

Primary immunodeficiency Neutropenic patients due to leukaemia and/or chemotherapy Invasive candidiasis in premature neonates due to immature immune system Children in intensive care who have been treated with broadspectrum antibiotics and/or abdominal surgery

Answer 455

Maybe just remember that candida has positive blood cultures whereas aspergillus doesn't Aso candida assocaited with birth canal Aspergillus is everywhere

Answer 456

Sepsis syndrome 2nd/3rd week of life Thrombocytopenia Hyperglycaemia

Answer 457

Prematurity Indwelling catheters Broad spectrum antibiotics Steroids H2 blockers Abdominal surgery

Answer 458

Not for use in children below a certain age such as 16 or 18 years old

Answer 459

Route of administration is different Medicine used for something else Medicines used at a different dose to that recommended Children below started recommended age limit Medicines without license, including those being used in clinical trials

Answer 460

Increased rate of ADRs Including death

Answer 461

•Neonates/infants are more **sensitive** to drugs than adults —due mainly to organ system immaturity * Neonates/infants are at increased risk for **adverse drug reactions** * Young patients show greater individual **variation** In the early post-natal period there is a higher incidence of therapeutic errors Adolescence: - sexual development - major changes in body size and coposition - affect drug metabolism, alterations in metabolism as a result of drinking smoking and taking drooogs 30% of prescribed medicines are off label in the early child Almost all medicines used during the early post-natal period are prescribed and used as off label Most commonly used drugs in children have a wide therapeutic index

Answer 462

Reduced gastric emptying, adult levels are reached at 3 years Absorption reaches adult values by 6-8 months Bioavailability of drugs is reduced if there is haigh hepatic clearance and a high first pass metabolism. There is also large variability in these drugs Drugs which rely on entero-hepatic circulation such as cyclosporin are also highly variable Decreased gastric emptying, decreased absorption, reduced bioavailability

Answer 463

Enhanced in infants and children, especially with damaged skin of an occlusive dressing

Answer 464

•Rectal –Used in patients who are vomiting or who are unwilling to take oral medication. –Avoids first-pass metabolism. –Not ideal as significant variation, few preparations, trauma.

Answer 465

–Newborn infants have high extracellular fluid volume of 45%. At one year 25%, and •Adults 20-25%.

Answer 466

–Total body water is high 75-92%. •Adults are about 50-60%.

Answer 467

–Fat content is low 12% in term infants, 30% at 1 year and •18% in the adult

Answer 468

* In terms of drug dosage this means that larger initial doses on a mg/kg body weight need to be given to achieve correct plasma concentration. * However after the loading dose the dosage interval may need to be increased or the daily dose decreased to compensate for the decreased hepatic function or decreased renal elimination. * ASSUMING THAT PHARMACODYNAMIC RESPONSE IS SIMILAR TO THE ADULT So bigger initial dose, space out later doses

Answer 469

Plasma binding is reduced in the neonate meaning that there is a greater amount of unbound or active drug

Answer 470

Not fully developed at birth Drugs have relatively easy access to the CNS

Answer 471

Longer –In the neonate liver metabolism is immature, thus drugs eliminated by the liver have a longer t1/2 –This results in a longer time to reach steady state (4xt1/2), an increase in steady state concentration –The same applies to drugs eliminated by the kidneys.

Answer 472

It is very slow in the neonatal period Neonates have high sensitivtiy to drugs that are cleared by hepatic metabolism Metabolic activity increases rapidly from about 1 month after birth with adult activity by 1 year of age

Answer 473

Hepatic metabolism is more rapid and half life is shorter

Answer 474

3-6 months tubular function at 12 months Consideration of renal function is most important in the neonate For most drugs T 1/2 is prolonged

Answer 475

Sensitvity to drugs is increased by fever dehydration Acidosis (decreased cellular penetration of basic drugs)

Answer 476

Fenestrated endothelial cell Glomerular basement membrane Podocyte with their slit diaphragms

Answer 477

Glomerular injury

Answer 478

1 - nephritic syndrome 2 - nephrotic syndrome

Answer 479

Glomerulr structural support Embedded in GBM Regulates the blood flow of the glomerular capillaries

Answer 480

Minimal change disease - results in nephrotic syndrome

Answer 481

**Dipstick** **Protein creatinine ratio, early morning urine is best.** (normal pr:cr ratio is less than 20mg/mmol) Nephrotic range is greater than 250mg/mmol **24 hour urine collection (gold standard)** Normal is less than 60 mg/m2/24 hours Neohrotic range is greater than 1g/m2/24 hours Check bloods - potential to have low albumin (probably high lipids as well)

Answer 482

* Typical Features * Age (2-5yrs) * Normal blood pressure * Resolving microscopic haematuria * Normal renal function * Atypical features * Suggestions of autoimmune disease * Abnormal renal function * Steroid resistance –Only then consider renal biopsy

Answer 483

Typical features: give prednisolone for 8 weeks

Answer 484

Steroids - causes the complete loss of proteinuria (if there is no response to steroids consider FSGS) Initial relapse is treated with further steroid course Subsequent relapses are treated with Cyclophosphamide Cyclosporin Tacrolimus Mycophenolate mofetil Rituximab

Answer 485

Podocyte loss Congenital causes are as a result malformation of nephrin and podocin proteins - also results in loss of podocytes

Answer 486

Glomerulonephritis Post infectious glomerulonephritis IGA / HSP (henoch schonlein purpura) UTI Trauma Stones

Answer 487

**Post infectious GN** **HSP / IgA nephropathy** Membranoproliferative GN Lupus nephritis ANCA positive vasculitis

Answer 488

ASO - antistreptolysin O - this is an antibody made in response to streptolysin O. The test would be called an ASOT - anti-streptolysin O titre Streptolysin O is produced by most A strains of Strep, many strains of groups C and G strep as well To rule out wegners you can test ANCA as well. C- ANCA = wegners, P ANCA = microscopic polyarteritis

Answer 489

Features ## Footnote general: headache, malaise haematuria proteinuria hypertension low C3 raised ASO titre

Answer 490

It is caused by immune complex (IgG, IgM and C3) deposition in the glomeruli. Young children most commonly affected.

Answer 491

Differentiating between IgA nephropathy and post-streptococcal glomerulonephritis ## Footnote post-streptococcal glomerulonephritis is associated with low complement levels main symptom in post-streptococcal glomerulonephritis is proteinuria (although haematuria can occur) there is typically an interval between URTI and the onset of renal problems in post-streptococcal glomerulonephritis

Answer 492

Antibiotic Support renal functions Diuretics for fluid overload

Answer 493

IgA nephropathy

Answer 494

Presentations ## Footnote young male, recurrent episodes of macroscopic haematuria typically associated with mucosal infections e.g., URTI nephrotic range proteinuria is rare renal failure is unusual and seen in a minority of patients

Answer 495

•Clinical diagnosis –Mandatory palpable purpura –one of 4 1. Abdominal pain 2. Renal involvement 3. Arthritis or arthralgia 4. Biopsy –IgA depostition

Answer 496

IgA vasculitis - affects small vessels IgA Nephropathy and IgA vasculitis with nephritis (HSP) – spectrum of same disease

Answer 497

•1-3 days post trigger –Viral URTI in 70% –Streptococcus, drugs •Duration of symptoms –4-6 weeks –1/3rd relapse •Nephritis –Mesangial cell injury

Answer 498

•**Treatment** –**Symptomatic** •Joints, gut (joints - heinoch shonelein syndrome causes arthritis, large overlap with IgA nephropathy) **–Glucocorticoid therapy** * Not helpful in renal disease * May help with gastrointestinal involvement **–Immmunosuppresion (cyclophosphamide)** •Trial in moderate to severe renal disease –Long term •Hypertension and proteinuria screening

Answer 499

Retention of urea Anuria/oliguria (less than 0.5 ml/kg/hr) Hypertension with fluid overload Rapid rise in plasma creatinine (serum creatinine is 1.5 times the age specific reference)

Answer 500

•Interpretation of AKI warning score: –AKI 1: Measured creatinine \>1.5-2x reference creatinine/ULRI –AKI 2: Measured creatinine 2-3x reference creatinine/ULRI –AKI 3: Serum creatinine \>3x reference creatinine/ULRI

Answer 501

Loss of volume (vomitting, diarrhoea, haemorrhage, dehydration) Hypotension and shock Congestive heart failure NSAIDS ans ACEi - these can rreduce blood flow to the kidneys

Answer 502

Acute tubular necrosis (consequence of hypoperfusion and drugs) Acute interstitial nephritis (NSAIDS - autoimmune) Glomerulonephritis HUS Other causes include: - Myeloma, intra-renal vascular obstruction, myeloma, thrombotic microangiopathy, rhabdomyolysis

Answer 503

Intraluminal (calculus, clot, sloughed papilla) Intramural (malignancy, ureteric stricture) Extramural - Malignancy

Answer 504

Post diarrhoea - enterohaemorrhagic E.Coli (VTEC or STEC) Pneumococcal infection Drugs Atypical HUS

Answer 505

Microangiopathic haemolytic anaemia Thrombocytopenia AKI

Answer 506

Reflux nephroapthy Dysplasia Obstructive uropathy (posterior urethral valves) These may be isolated or part of a much bigger clinical picture (turner, trisomy 21, brachio-oto-renal, prune belly syndrome)

Answer 507

Cystic kidney disease Cystinosis

Answer 508

Oliguria Peripheral oedema Uremia GI ulcerations, bleeding and diarrhoea Encephalopathy (fatigue, appetite loss, confusion) Increase potassium (cardia arrhythmias) Anaemia - low erythropoeitin production by the kidneys

Answer 509

Fever Vomiting Lethargy Irritability (can also cause poor feeding, failure to thrive, abdominal pain, jaundice, haematuria, offensive urine)

Answer 510

Urine collection method ## Footnote clean catch is preferable if not possible then urine collection pads should be used cotton wool balls, gauze and sanitary towels are not suitable invasive methods such as suprapubic aspiration should only be used if non-invasive methods are not possible

Answer 511

Diptick - leucocyte esterase activity, nitrites, unreliable under the age of 2 Microscopy: Pyuria over 10 WBC per cubic mm, bacturia Culture: greater than 10^5 colony forming units

Answer 512

1-ureter only 2-ureter, pelvis, calyces 3-dilatation ureter 4-Moderate dilatation of ureter ± pelvis ±tortuous ureter, obliteration of fornices 5-gross dilatation/tortuosity, no papillary impression in calyces

Answer 513

Ultrasound DMSA (scarring/function) Micturating cystourethrogram MAG 3 scan

Answer 514

•Lower tract –3 days oral antibiotic •Upper tract / pyelonephritis –antibiotics for 7-10 days •Oral if systemically well –Role of prophylaxis ?? •Prevention –Fluids, hygiene, constipation –Voiding dysfunction Oral antibiotics are given from 3 months of age: trimethoprim, co-amoxiclav and cephalosporin If using IV - 3rd generation cephalosporin or co-amoxiclav, IV aminoglycosides effective Pass Medicine: infants less than 3 months old should be referred immediately to a paediatrician children aged more than 3 months old with an upper UTI should be considered for admission to hospital. If not admitted oral antibiotics such as cephalosporin or co-amoxiclav should be given for 7-10 days children aged more than 3 months old with a lower UTI should be treated with oral antibiotics for 3 days according to local guidelines, usually trimethoprim, nitrofurantoin, cephalosporin or amoxicillin. Parents should be asked to bring the children back if they remain unwell after 24-48 hours antibiotic prophylaxis is not given after the first UTI but should be considered with recurrent UTIs

Answer 515

Late referral Hypertention Proteinuria High intake of protein, phosphate and salt (these are all things that are avoided in CKD) Acidosis Recurrent UTIs

Answer 516

post-dysentery - classically E coli 0157:H7 ('verotoxigenic', 'enterohaemorrhagic') tumours pregnancy ciclosporin, the Pill systemic lupus erythematosus HIV

Answer 517

Investigations ## Footnote full blood count: anaemia, thrombocytopaenia, fragmented blood film U&E: acute renal failure stool culture

Answer 518

Management ## Footnote treatment is supportive e.g. Fluids, blood transfusion and dialysis if required there is no role for antibiotics, despite the preceding diarrhoeal illness in many patients the indications for plasma exchange in HUS are complicated. As a general rule plasma exchange is reserved for severe cases of HUS not associated with diarrhoea

Answer 519

Simple ## Footnote –Developmental * Dysplasia (multicystic dysplastic kidney - non-functioning kidney, hypertrophy of the contralateral kidney) * Multicystic dysplastic –Genetic * Autosomal Recessive (ARPKD) (potter's sequence - large bright kidneys, oligohydramnios, pulmonary hypoplasia, fetal compression of faces, contracture) defect in chromosome 6 that codes for fibrocystin. Fibrocystin is needed for normal renal tubule development. End stage renal failure develops in childhood. * Autosomal Dominant (ADPKD) - most common inherited form of kidney disease - managment with tolvaptan. Autosomal dominant is more common than recessive. •Syndromic –Various forms of Juvenile Familial Nephronophthisis (JFN) –Acquired •Cancer

Answer 520

Good to know that JFN is normotensive whereas the polystic disorders are hypertensive PKD 1 is more aggressive than PKD 2

Answer 521

X - linked dominant condition Defect in gene coding for type 4 collagen

Answer 522

The glomerular basement membrane

Answer 523

Alport's syndrome usually presents in childhood. The following features may be seen: ## Footnote microscopic **haematuria** progressive **renal failure** bilateral **sensorineural deafness** **lenticonus**: protrusion of the lens surface into the anterior chamber **retinitis pigmentosa** renal biopsy: splitting of lamina densa seen on electron microscopy

Answer 524

Greater area:volume ratio Immature shivering function

Answer 525

Obstruction - birth asphyxia, croup, epiglottiis (caused by haemophilus influenzae), bronchiolitis, asthma, pneumothorax Depression - poisoning, convulsions, raised ICP (head injury, acute encephalopathy from meningitis or encephalitis)

Answer 526

Fluid loss (gastroenteritis, burns, trauma) Fluid malabsorption: - Sepsis - Anaphylaxis - Heart failure

Answer 527

1 - death instantaneously, unsurvivable major vessel and brain injury 2 - Death from ABCD problems 3 - multi-organ failure, sepsis

Answer 528

Symptoms Baby pyrexia or hypothermia Poor feeding Lethargy Early jaundice Hypoglycaemia Hyperglycaemia Asymptomatic

Answer 529

Premature rupture of membranes Maternal pyrexia Group B strep in the mother

Answer 530

Admit NNU Partial septic screen (FBC, CRP, blood cultures) and blood gas Consider CXR, LP IV penicillin and gentamicin 1st line 2nd line iv vancomycin and gentamicin Add metronidazole if surgical/abdominal concerns Fluid management and treat acidosis Monitor vital signs and support respiratory and cardiovascular systems as required

Answer 531

Group B strep E.Coli Listeria Coag - neg staphylococco (if lines insitu) Haemophilus influenzae

Answer 532

Early onset - birth to 1 week Late onset or recurrence up to 3 months Symptoms - may be non-specific May be non-specific May have no risk factors **Complications**: - **Meningitis, DIC, pneumonia and respiratory collapse, hypotentison and shock** Prognosis - 4 to 30% mortality

Answer 533

Rubella: Sensorineural deafness Congenital cataracts Congenital heart disease (e.g. patent ductus arteriosus) Glaucoma Toxoplasmosis: Cerebral calcification Chorioretinitis Hydrocephalus CMV: Growth retardation Purpuric skin lesions They all cause hepatosplenomegaly

Answer 534

May cause pregnancy loss or preterm delivery

Answer 535

Characteristic rash on hands and feet

Answer 536

Sepsis Transient tachypnoea of the newborn (most common cause of respiratory distress in newborns - caused by failure to clear fetal lung fluid) Meconium aspiration

Answer 537

Presents within the 1st few hours of life Clinically seen as grunting, tachypnoea, oxygen requirement, normal gases

Answer 538

It is self-limiting and common Managment is supportive, antibiotics, fluids, O2, airway support

Answer 539

Post dates (aged placenta) Maternal diabetes Maternal hypertension Difficult labour

Answer 540

Cyanosis Increased work of breathing grunting apnoea floppiness

Answer 541

Blood gas Septic screen CXR

Answer 542

Suction below cords Airway supoprt - intubation and ventilation Fluids and antibiotics IV Surfactant NO or ECMO

Answer 543

•Cyanosis occurs when there is more than 5g/dl of deoxyhaemoglobin Respiratory and sepsis is more common than cardiac

Answer 544

Examination and history Sepsis screen Blood gas and blood glucose CXR Pulse oximetry ECG Echo Hyperoxia test

Answer 545

TGA Tetralogy of fallot TAPVD Hypoplastic left heart syndrome Tricuspid atresia- absence of tricuspid valve and therefore absence of right atrioventricular connection - right atrium is hypoplastic Truncus arteriosus (pulmonary trunk and aorta are one vessel) Pulmonary artresia (meconium aspiration was previously mentioned as causing cyanosis)

Answer 546

May require admission to NNU Monitor blood glucose Start IV 10% glucose Increase fluids Increase glucose concentration (central IV access) Glucagon Hydrocortisone Role of hydrocortisone: Cortisol is needed to maintain blood glucose levels Cortisol is permissive to glucagon Stimulates the formation of gluconeogenic enzymes - enhancing gluconeogenesis Proteolysis - cortisol stimulates the breakdown of muscle protein to provide gluconeogenic substrates for the liver Lipolysis in adipose tissue releases FFA - preserves glucose concentration and also provdes moresubstrate for gluconeogenesis Decreases insulin sensitivity of muscles and adipose tissue.

Answer 547

Admit and place in incubator Sepsis screen and antibiotics Consider checking thyroid function Monitor blood glucose

Answer 548

Placental problem Long difficult delivery Umbilical cord prolapse Infectoin Neonatal airway problem Neonatal anaemia

Answer 549

1st - within minutes without O2 - Cell damage occurs with lack of blood flow and O2 2nd - Reperfusion injury - Can last days or weeks - Toxins are released from damaged cells

Answer 550

Treat seizures Therapeutic hypothermia Support Fluid restriction (avoid cerebral oedema) Monitor for renal and liver failure Respiratory support Cardiac support

Answer 551

Constipation Large bowel artresia Imperforate anus Hirschsprungs disease (a congenital condition in which the rectum and part of the colon fail to develop a normal system of nerves, leading to an accumulation of faeces in the colon following birth.) Diagnostic test is rectal biopsy Meconium ileus (assocaited with cystic fibrosis)

Answer 552

**• 1st 24hrs :** * Haemolytic ( G6PD – spherocytosis ) * TORCH ( congenital infection ) Also included in this section is haemolysis (rhesus incompatibility, other antibodies, ABO incompatibility)infection, hereditary anaemias **• 2nd day – 3rd wk :** * Physiological ( gone after 1st wk ) * Breast milk * **Sepsis** * **Polycythaemia** * Cephalhaematoma **•Crigler-Najjar Syndrome** •Haemolytic disorders **• After 3rd wk :** * Breast milk * Hypothyroidism * Pyloric stenosis * Cholestasis NHS: ABO bloud group mismatch between mum and baby Rhesus factor disease Urinary tract infection Crigler najjar syndrome Blockage in the bile ducts and gall bladder G6OD deficiency?

Answer 553

Continue breast feeding If the child is receiving treatment, make sure to give the child more fluids and more regular feeds

Answer 554

Polycythaemia (or it could be SVCS)

Answer 555

* 30 – 70% of normal term neonates. * very rare in the pre-term.

Answer 556

Maculo-papular

Answer 557

Rash fades by the end of the first week no treatment is reqiured

Answer 558

Treat underlying cause Hydrate Phototherapy Exchange transfusion Immunoglobulin

Answer 559

**Limited glucose supply** - premature babies - perinatal stress * *Hyperinsulinsim** (infants of diabetic mothers) * *Increased glucose utilisation** - Small and large for gestational age Hypothermia Sepsis

Answer 560

Jitteriness **Hypothermia** Temperature instability **Lethargy** Hypotonia Apnoea, irregular respirations Poor suck / feeding **Vomiting** High pitched or weak cry **Seizures** Asymptomatic

Answer 561

Defined as blood sugar below 2.6 mmol/l

Answer 562

at low or high levels When there is poor perfusion When there is polycythaemia

Answer 563

Low birth weight Babies requiring prolonged resuscitation

Answer 564

Dry quickly Remove wet linens Use warm towels/blankets Provide radiant warmer heat Use heated / humidified oxygen

Answer 565

Restriction of the tongue beyond the alveolar margins OR Heavy grooving of the tip of the tongue OR Feeding is affected

Answer 566

Maxillary and medial nasal processes fail to merge, usually around 5 weeks gestation

Answer 567

Complete vs incomplete unilateral vs bilateral Cleft palate

Answer 568

Feeding issues ◦Special bottles and teats ◦Can still attempt breast feeding Airway problems Associated anomalies ◦Need **hearing** screen ◦Need **cardiac** echo ◦Remember trisomies

Answer 569

Cataracts - treated with lens removal and artifical lens Retinoblastoma - laser therapy, chemo, surgical removal of eye

Answer 570

Spina bifida Possible kidney problem

Answer 571

Localised swelling over one or both sides of the head Becomes maximal by the 3rd to 4th day of life Soft, non-translucent swelling Limited to one of the cranial bones, usually the parietal bone DOES NOT CROSS JOINT LINES

Answer 572

Haemorrhage beneath the pericranium No assocaition with intracranial bleeding

Answer 573

No treatment is required Resolution occurs in 3-4 weeks occasionally, if the haematoma is very large, the increased haemolysis results in increased or prolonged neonatal jaundice

Answer 574

Caput succedaneum Does not cause complications and resolves over the first few days

Answer 575

Medial (varus) or lateral (valgus) deviation of the foot is often positional and requires no treatment other than **physiotherapy** Fixed talipes requires more vigorous manipulation, **strapping**, **casting** or possibly surgery Babies with significant talipes may also have developmental dysplasia of the hips

Answer 576

Goal ◦Relocate head of femur to acetabulum so hip develops normally Pavlik harness Surgical reduction

Answer 577

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

Answer 578

Indicates lack of maturity

Answer 579

Tall forejead Polydactyly Syndactyly

Answer 580

Greig / GL13

Answer 581

Very similar to treacher collins (treacher collins is autosomal dominant though) Micrognathia Posterior displacement of the tongue (may result in upper airway obstruction) Cleft palate

Answer 582

Turner's syndrome is a chromosomal disorder affecting around 1 in 2,500 females. It is caused by either the presence of only one sex chromosome (X) or a deletion of the short arm of one of the X chromosomes. Turner's syndrome is denoted as 45,XO or 45,X Features ## Footnote short stature shield chest, widely spaced nipples webbed neck bicuspid aortic valve (15%), coarctation of the aorta (5-10%) primary amenorrhoea cystic hygroma (often diagnosed prenatally) high-arched palate short fourth metacarpal multiple pigmented naevi lymphoedema in neonates (especially feet) gonadotrophin levels will be elevated

Answer 583

C - Cardiac abnormalities A - Abnormal facies T - Thymic aplasia C - Cleft palate H - Hypocalcaemia/ hypoparathyroidism 22 - Caused by chromosome 22 deletion Also causes loss of T cell function