Paediatrics Flashcards

Question

Earliest presentation of CF in neonates?

Answer 1

Meconium ileus - Meconium isnt passed and instead causes a blockage in the intestine.

Answer 2

- Newborn guthrie heel prick screening (Immunoreactive trypsinogen) - GOLD: Chlorine sweat test. Pilocarpine induces sweating. Sweat has more chlorine (>60mmol/L) - Faecal elastase (pancreatic insufficiency)

Answer 3

Life expectancy <40 CF related diabetes Liver/biliary cirrhosis Recurrent URTI Malabsorption - Delayed puberty - Osteoporosis - Infertility

Answer 4

Inflammation of the middle ear (between tympanic membrane and oval window). Most commonly secondary to viral URTI (RSV and rhinovirus) but ear infection is bacterial (S. pneumoniae, Moraxella catharallis, H influenzae, S aureus)

Answer 5

Usually self limiting (3 days) Give Abx if: - >4 days and no improvement - Systemically unwell - immunocompromised - If under 2 and bilateral otitis media - Discharge or perforation

Answer 6

Otitis Media with Effusion. Chronic ear infection or eustachian tube dysfunction can cause a build up on viscous inflammatory fluid, causing conductive hearing loss. Unilateral OME can suggest a middle ear tumour.

Answer 7

Most cases resolve in 3 months. Otherwise, non surgical (hearing aid, autoinflation) or surgical intervention may be needed (Myringotomy and grommet insertion)

Answer 8

Negative pressure in middle ear causes fluid accumulation leading to glue ear. A grommet is a small pipe that is placed into the tympanic membrane to keep pressure the same on either side by allowing air to enter the middle ear. It is inserted after a myringotomy (incision in tympanic membrane to drain fluid in middle ear).

Answer 9

6-12 months, but some can be long term. Avoid water! and regular ENT check ups

Answer 10

Conductive - When sound waves not conducted through outer ear to eardrum. Causes: eardrum perforation, fluid in middle ear, earwax buildup. Sensorineural - Damage to inner ear (cochlea) or to nerve pathways from ear to brain. Causes: Aging, loud sound exposure, Meniere's disease.

Answer 11

Conductive - Rinnes: Bone conduction > Air conduction (negative result) - Webers: Heard more in the bad ear Sensorineural - Rinnes: Air conduction > Bone conduction (Positive - Normal result) - Webers: Heard more in the good ear

Answer 12

Ear wax, otitis media, otitis externa

Answer 13

Autosomal dominant replacement of normal bones in ear with spongy vascular bone. - Conductive deafness - Tinnitus - "Flamingo tinge" to tympanic membrane - Family history

Answer 14

Bronchoconstriction in response to inflammation and oedema following an RSV or Rhinovirus infection

Answer 15

Preschoolers <3 years SABA, nebulised ipratroprium bromide or 5 days oral prednisolone

Answer 16

Infection of fat and muscle cells posterior to orbital septum. Commonly due to URTI from sinuses and is a medical emergency with high mortality. Caused by S aureus, HiB and streptococcus.

Answer 17

- Childhood (7-12) - Previous sinus infection - Lack of Hib immunisation - Recent eyelid infection - Ear/face infection

Answer 18

Periorbital/preseptal does not have reduced visual acuity or pain with eye movement.

Answer 19

CT with contrast - look for inflammation of orbital tissue

Answer 20

Hospital admission and 7-10 days of IV antibiotics - Cefotaxime

Answer 21

Preseptal is when the infection is anterior to orbital septum. Includes eyelids, skin and subcutaneous tissue but does not affect the orbit or its contents. More common in kids under 21 months

Answer 22

Eyes pointing different directions AKA Strabismus - Misalignment of the visual axes. Can be concominant (imbalance in extraocular muscles - Convergent more common than divergent) or because one is paralysed (rare, due to paralysis of extraocular muscles) Detected using corneal light reflex to check symmetry of light reflection

Answer 23

- Louder than 2/6 - Diastolic - Louder standing - Symptoms (failure to thrive, feeding difficulty, cyanosis, SOB)

Answer 24

Mitral regurgitation - Mitral (5th intercostal, mid clavicular) Tricuspid regurgitation - Tricuspid (5th intercostal, left sternal edge) Ventricular septal defect - Left lower sternal border

Answer 25

Aortic stenosis - Aortic (2nd intercostal, right sternal edge) Pulmonary stenosis - Pulmonary (2nd intercostal left sternal edge) Hypertrophic obstructive cardiomyopathy - (4th intercostal left sternal edge - just above tricuspid)

Answer 26

Abnormal connection between descending aorta and pulmonary artery. Usually close with first breaths. Aorta has greater pressure than pulmonary vessels, causes a left to right shunt, causing pulmonary hypertension and right heart strain and RVH. Increased pressure then causes LVH - Prematurity - Born at high altitude - Maternal rubella infection

Answer 27

Continuous crescendo-decrescendo "machinery" murmur. - Left subclavicular thrill - Large volume, bounding, collapsing pulse - Wide pulse pressure - Apex heave

Answer 28

Indomethacin or ibuprofen - usually closes the connection in neonates. Given after 1 week, to allow time to close on its own Monitoring until 1yo

Answer 29

Mid (AKA ejection) systolic, crescendo-decrescendo murmur loudest at upper left sternal edge. Also has a fixed split heart sound (doesn't change with breathing)

Answer 30

Blood shunts from left atrium to right. Hence, blood flows to lungs (ACYANOTIC) but increased right heart flow causes right heart overload and strain. Can lead to right heart failure and pulmonary HTN. Most common congenital heart defect in adults, 50% dead by 50

Answer 31

Pulmonary HTN increases so much the pulmonary pressure > systemic pressure. Reverses the L2R shunt and causes it to become R2L, so blood bypasses lungs and becomes cyanotic.

Answer 32

Ostium Secondum (70%, associated with Holt-Oram syndrome (tri-phalangeal thumbs)) Ostium primum (associated with abnormal AV valves)

Answer 33

Most common congenital heart disease. 50% close spontaneously. - Congenital VSDs are associated with chromosomal disorders (Down's, Edwards, Pataus etc) and congenital infections.

Answer 34

In utero (20 wk scan). Post natally, failure to thrive, features of heart failure, pan systolic murmur.

Answer 35

Most common CHD at birth. Cyanotic heart disease in which aorta leaves the right ventricle and pulmonary artery leaves left ventricle. (positions switched). Causes oxygenated blood to flow round the lungs, and deoxygenated blood to flow around the body

Answer 36

- Loud single S2 - Prominent RV impulse - "egg-on-side" appearance on CXR

Answer 37

VORP - cause right to left cardiac shunt; blood bypasses lungs. Ventricular Septal Defect - Blood shunts between ventricles. Oxygenated and deox. mix. Deox more into left than ox into right. Overriding Aorta - Aorta further right than normal. RV sends deox blood into it RV Hypertrophy - Due to added resistance of LV, ensures deox blood is shunted to left, rather than the other way. Pulmonary stenosis - RV outflow obstruction makes it harder for deox blood to reach lungs

Answer 38

4 congenital abnormalities causing an increase in deoxygenated blood around the body. Causes cyanosis, and the child may develop hypercyanotic "tet" spells with increased need (crying, feeding etc), causing a "squatting position", resp distress, syncope, tachypnoea.

Answer 39

- Family history - Diabetic mum - Down's - Alcohol in pregnancy - Rubella

Answer 40

X - Boot shaped heart Echo - RV hypertrophy

Answer 41

Surgical repair Tet spells - Beta blockers, oxygen, morphine, sodium bicarbonate, phenylephrine

Answer 42

Pulmonary valve doesn't form properly, meaning blood cant reach lungs. Requires alternative pathway to lungs.

Answer 43

Degenerative hip joint condition affecting 4-8yo. Due to avascular necrosis of the femoral head (femoral epiphyses), followed by revascularisation and reossification over 18-36 months. 5x more common in boys

Answer 44

Most resolve with conservative management, e.g. bracing to keep femoral head in place. If >6 yo, or severe deformity, surgery. - Osteoarthritis - Premature fusion of growth plates

Answer 45

Type 2 hypersensitivity to a recent strep pyogenes infection (strep throat - 2-4 weeks after). Rare in west, causing joint pain and carditis. Repeat exposure can cause fibrosis of valves, causing regurgitations

Answer 46

Aschoff bodies (granulomatous bodies)

Answer 47

Revised Jones criteria (JONES-FEAR) Evidence of recent infection (group A antigen test, positive throat culture, strep antibodies (ASO antibody titre)) + 2 major signs or 1 major 2 minor. - Joint arthritis - Organ inflammation (carditis+murmur) - Nodules under skin (firm, painless) - Erythema marginatum (red splodgy rash all over) - Sydenham's chorea - Fever - ECG (prolonged PR) - Athralgia without arthritis - Raised ESR/CRP

Answer 48

Acute - Mitral and aortic regurgitations Chronic - Mitral stenosis

Answer 49

Oral penicillin V for 10 days and NSAIDs

Answer 50

Commonest cause of vomiting in infancy. Normal to regurgitate feeds but GORD when it starts to cause distress. Caused by a weak sphincter which normally resolves by 1 year

Answer 51

Signs of distress more than usual post feed vomiting - Chronic cough - Hoarse, excessive crying, especially while feeding - Milky vomits and regurgitation after feeds or after being laid flat - Poor weight gain

Answer 52

- Not keeping feeds down +- projectile/forceful vomiting (pyloric stenosis or intestinal obstruction) - Bile stained vomit and/or abdominal distention (intestine obstruction) - Haematemesis or malaena (peptic ulcer, oesophagitis, varices) - Respiratory symptoms (aspiration/infection) - Rash, angioedema (milk protein allergy) - Apnoeas

Answer 53

Usually doesn't need medical therapy. - 30 degree head up position during feeds - Sleep on back - Ensure not overfeeding, and burp regularly - Thickened formula - Gaviscon with feeds if bad

Answer 54

A rare condition causing brief episodes of abnormal movements usually associated with GORD - Torticollis: Forceful contraction of the neck muscles causing twisting of the neck - Dystonia: Abnormal muscle contractions causing twisting movements, arching of the back. Usually self resolves but important differential is infantile spasms and seizure

Answer 55

Usually presents in 2nd to 4th weeks of life, with - Projectile vomiting (~30mins after a feed) - Constipation and hydration - Palpable mass in upper abdo - Hypochloraemic, hypokalaemia alkalosis due to persistent vomiting. Caused by hypertrophy of the circular muscles of the pylorus

Answer 56

Diagnosed with ultrasound Managed with Ramstedt pyloromyotomy

Answer 57

A sensitivity to the gluten protein. Repeated exposure leads to villous atrophy causing malabsorption. Children normally present before 3yo. Normally affects small bowel, particularly the jejunum

Answer 58

Often asymptomatic, low threshold for testing - Failure to thrive - Diarrhoea - Abdominal distention - Anaemia - Mouth ulcers - Dermatitis herpetiformis - itchy, blistering skin rash that typically appears on the abdomen

Answer 59

HLA-DQ2 (90%) HLA-DQ8

Answer 60

Antibodies - Anti-TTG and Endomysial antibodies (IgA - undetectable if IgA deficiency) - Anti-DGPs (Deaminated Gliadin Peptides) Endoscopy and intestinal - Crypt hyperplasia - Villous atrophy - Increased intraepithelial lymphocytes - Lamina propia infiltration Gluten free diet trial

Answer 61

Gluten free diet

Answer 62

Autoimmune - T1DM - Thyroid disease - Autoimmune hepatitis - PBC/PSC - Down's

Answer 63

Idiopathic or functional - no underlying cause Secondary: - Hypothyroidism - Hirschsprung's Disease - Cystic fibrosis - Hypercalcaemia - Dehydration - Low fibre diet - Medication induced e.g. opiates

Answer 64

- Less than 3 stools a week - Hard stool difficult to pass - Rabbit dropping (bristol stool type 1) - Abdominal pain, waxes and wanes with passage of stool. Possibly a palpable abdominal mass - Overflow soiling - Poor appetite - Straining, pain, bleeding Retentive posturing - Straight legged - Tiptoed - Back arched

Answer 65

- Reported from birth or first weeks of life - Meconium took >48 hours - Ribbon stool pattern - Weakness in legs - Faltering growth - Distension - Disclosure or evidence of mistreatment

Answer 66

4 years old. Usually a sign of chronic constipation, causing stretching and desensitisation of the rectum.

Answer 67

- Start after first few weeks of life, obvious precipitating factors - Meconium in less than 48h - Growth normal - No neurological deficit or problems - Changes in diet

Answer 68

Diagnosis of idiopathic can be made if no red flags - Movicol Paediatric Plan first line (contains Polyethylene glycol 3350 and electrolytes) - Add stimulant laxative if no disimpaction (Senna) - Lifestyle advice: High fibre, hydration, exercise, reduce risk factors If Impaction: - May need disimpaction regime with high dose of laxatives <6 months - Bottle fed: Water between feeds, gentle massaging and bicycling legs - Breast fed: Unusual to be constipated when breast fed

Answer 69

Encourage toilet visits - Scheduling visits - Bowel diary - Star charts

Answer 70

Rotavirus Norovirus Highly common and contagious Adenovirus possible but more subacute

Answer 71

E coli is a normal intestinal bacteria. Spread through contact with contaminated faeces, unwashed salad and contaminated water. E coli 0157 produces shiga toxin, causing cramps, bloody diarrohea, vomiting. Shiga toxin destroys bloody cells and leads to haemolytic uraemic syndrome. Abx increase risk of HUS so avoid if E coli.

Answer 72

Travellers diarrhoea. Most common cause of bacterial gastroenteritis worldwide. Gram negative, curved/spiral Spread through - Raw/undercooked chicken - Untreated water - Unpasteurised milk Incubation 2-5 days and 3-6 days of symptoms - Abdominal cramps - Bloody diarrhoea - Vomiting and fever Azithromycin or ciprofloxacin if severe

Answer 73

Spread through faeces contaminated drinking water, swimming pools, food. Incubation 1-2 days, symptoms usually resolve within a week without treatment. Bloody diarrhoea, cramps, fever. Shigella produces the shiga toxin that causes HUS. Azithromycin or ciprofloxacin in severe cases

Answer 74

Spread through raw eggs or chicken, or food contaminated with small animal poo. Symptoms usually resolve in a week. Watery diarrhoea that can be associated with blood or mucus, abdo pain, vomiting. Abx only in severe cases

Answer 75

Bacillus Cereus - Fried rice left at room temp. Sx start in 5 hours, resolves in 24 hours. Vomiting first and diarrhoea. Yersinia Entercolitica - Gram negative bacillus, spread through pork. Affects children. Key symptom is lymphadenopathy. In adults this causes right sided mesenteric lymphadenopathy (mimicking apendicitis) Giardiasis - Microscopic parasite. Live in small intestine of mammals, releasing cysts in their stools, which contaminate food and water. Treated with metronidazole

Answer 76

Produces enterotoxins on food such as eggs, dairy and meat. Cause intestine inflammation, causing diarrhoea, profuse vomiting, abdo cramps and fever. Sx settle in 12-24 hours

Answer 77

Cows' milk intolerance

Answer 78

Jittery movements Increased muscle tone Hyperreflexia Convulsions Drowsiness/coma

Answer 79

- 50ml/kg low osmolarity oral rehydration solution over 4 hours.

Answer 80

Abdominal pain or Bloating or Change in bowel habit and stool constitution (watery, loose, hard, mucus) Pain relieved with defecation. >6 months. Diagnosed through exclusion and if - Altered stool passage - Abdominal bloating - Symptoms made worse by eating - Passage of mucus

Answer 81

- Rectal bleeding - Unexplained weight loss - FH of bowel or ovarian cancer - >60y onset

Answer 82

- FBC (check anaemia) - Inflammatory markers - Coeliac serology - Faecal calprotectin - CA125 (ovarian cancer) CA19/9 (pancreatic) *Ovarian presents similar, just much older. E.g. 50yo woman with bloating, abdo pain. Non specific

Answer 83

Lifestyle advice - Fluids - Regular small meals - Low FODMAP diet - Limit caffeine, alcohol, fatty foods - Reduce stress - Change fibre depending if constipation or diarrhoea CBT may be used

Answer 84

Appendix sits at end of caecum. Trapped stool or other obstruction causes infection and inflammation, which quickly causes gangrene and rupture. Rupture releases contents into abdomen, causing peritonitis and possibly sepsis.

Answer 85

- Severe central abdominal pain that moves to RIF over time. - Tenderness in McBurney's point (2/3 of the distance from belly button to ASIS (Anterior Superior Iliac Spine)) - Rovsing's sign (palpation of left iliac fossa causes pain in RIF) - Guarding Rebound tenderness and percussion tenderness if progression to peritonitis

Answer 86

Usually made clinically: - Pain moving centrally to RIF - low grade pyrexia - minimal vomiting CT GOLD Standard Ultrasound in women (rule out ovarian cancer and gynae pathology) Diagnostic laparoscopy if negative, with appendectomy in same procedure

Answer 87

Ectopic pregnancy - bHCG will be raised (test to rule out) Ovarian cysts - Pelvic and IF pain, not always right, can rupture or torsion Meckels diverticulum - Malformation of distal ileum, usually asymptomatic but can inflame, rupture, bleed, volvulus, intussusception. Mesenteric adenitis - Inflamed lymph nodes. Often associated with tonsilitis or URTI Appendix mass - Omentum surrounds and sticks to inflamed appendix. Treated with abx and supportively. Appendectomy once acute infection resolves

Answer 88

When a portion of the bowel invaginates into the lumen on the adjacent bowel, most commonly around the ileo-caecal region. This increases bowel size and narrows lumen, causing ostruction. Usually affects infants 6-24 months. Boys 2x as often

Answer 89

- Intermittent severe crampy progressive abdo pain - Inconsolable crying - During attack the infant will raise knees up and turn pale - Vomiting - Bloodstained stools "red-currant jelly" late sign - Sausage shaped mass in upper right quadrant May present with viral URTI preceding illness

Answer 90

Ultrasound is investigation of choice - target like mass - Managed with reduction by air insufflation. - OR Barium enema

Answer 91

Obstruction Gangrene Perforation Death

Answer 92

Congenital diverticulum of the small intestine. Remnant of omphalomesenteric duct and contains ectopic ileal, gastric or pancreatic mucosa Usually asymptomatic but can present with abdominal (RLQ) pain, rectal bleeding or intestinal obstruction. It is the biggest cause of painless massive GI bleeding between 1 and 2y.

Answer 93

Meckels scan - 99m Technetium pertechnetate scan, which has an affinity for gastric mucosa Managed - Laparoscopic surgery or diverticulectomy. - Small bowel resection and anastomosis.

Answer 94

Meckel's diverticulum - 2% of population - 2 inches long - 2 feet from ileocaecal valve - 2x as likely in boys - 2yo when it causes GI bleed - 2 types of ectopic tissue (pancreatic and gastric (susceptible to ulcer))

Answer 95

Congenital absence of nerve cells of the parasympathetic myenteric plexus are absent in the distal bowel and rectum. Known as Auerbach's and Meissner plexuses. Causes uncontrolled peristalsis, effectively being an obstruction.

Answer 96

3x more common in males Downs syndrome Neurofibromatosis Waardenburg syndrome

Answer 97

Delay in passing meconium Chronic constipation Abdo pain/distension Vomiting Poor weight gain and failure to thrive

Answer 98

Abdominal X ray Rectal biopsy GOLD Managed - Rectal washout/bowel irrigation - Definitive: Surgery to affected colon segment

Answer 99

Hirschsprung-Associated Enterocolitis Inflammation and obstruction of the bowel. 2-4 weeks of birth with fever, abdo distension, bloody diarrhoea, features of sepsis. Can lead to toxic megacolon and perforation IV Antibiotics, fluid resus, decompression of bowel

Answer 100

Blockage or absence of extrahepatic bile duct, causing jaundice due to high conjugated bilirubin, as it is not being excreted in the bowel.

Answer 101

Type 1: Proximal ducts present, but common duct obliterated Type 2: Atresia of the cystic duct, and systic structures found in porta hepatis Type 3: Atresia of left and right ducts to the level of the porta hepatis (most common)

Answer 102

- Jaundice extending beyond physiological 2 weeks - Dark urine and pale stools - Appetite and growth disturbance - Hepatosplenomegaly

Answer 103

Surgical - Kasai portoenterostomy (attach small intestine to liver opening, allowing bile excretion) - Progressive liver disease - Cirrhosis - HCC If surgery unsuccessful, may need transplant

Answer 104

First 24 always pathological - Rhesus haemolytic disease - ABO haemolytic disease - Hereditary spherocytosis - G6PD Deficiency 2-14 days - Usually physiological and associated with breastfeeding

Answer 105

Usually seen in the first 3 months of formula fed children, can rarely be seen in exclusively breastfed infants. CMPA for immediate (<2 hours, IgE mediated) CMPI if mild/moderate and delayed (several days, non IgE mediated)

Answer 106

Occurs in first 3 months of life: - Regurgitation and vomiting - Diarrhoea - Urticaria - Colic symptoms (crying, irritability) - Wheeze, chronic cough - Rarely anaphylaxis Cows milk protein intolerance has the bloating, wind, diarrhoea and vomiting, but no rash, angio-oedema, sneezing or coughing.

Answer 107

skin prick/patch testing total IgE and specific IgE (RAST) for Cows milk protein Avoiding cows milk should fully resolve issues

Answer 108

Avoid cow's milk If formula fed - Extensive hydrolysed formula first line replacement - If intolerance, can be started on milk ladder Breast feeding - Breast feeding mother should avoid dairy products - May need maternal calcium supplements - Hydrolysed formula milk when breastfeeding stops until 12 months, for at least 6 months

Answer 109

- Meconium ileus - Hirschprung's disease - Oesophageal/duodenal atresia - Intussusception - Intestinal malrotation

Answer 110

Abdominal X ray - Dilated loops of bowel proximal to obstruction and collapsed distal. - Absence of air in rectum

Answer 111

Chronic, non specific diarrhoea, between 1 and 4 years of age. Is a diagnosis of exclusion

Answer 112

Frequent, poorly formed, offensive stools Food material easily recognisable Child is normally well, active and has unimpaired growth. Normal appetite and normal/increased fluid intake. No positive findings on examination or lab investigations

Answer 113

Paternal reassurance, avoidance of full strength fruit juice. Faeces normally becomes firm when child is toilet trained or by 3 years

Answer 114

Poor physical growth and development Faltering growth if they fall in centile spaces (e.g. distance between 75th and 50th centile) - 1 if birthweight <9th centile - 2 if birthweight 9-91st centile - 3 if birthweight above 91st centile

Answer 115

Inadequate intake - Maternal absorption - Neglect - Poverty Difficulty feeding - Poor suck (cerebral palsy) - Cleft lip/palate - Pyloric stenosis Malabsorption Increased energy requirements - Hyperthyroid - Cystic fibrosis, heart disease - Chronic infection Inability to process nutrition

Answer 116

- Height more than 2 centiles below mid parental height centile - BMI below 2nd centile

Answer 117

Chronic inflammation of the entire GI tract (mouth to anus). Terminal ileum and colon most commonly affected. Affects white people more

Answer 118

Family history Smoking White people OCP Diet low in fibre NSAID

Answer 119

NOD2/CARD15 gene mutation

Answer 120

Transmural inflammation with areas of healthy bowel in between known as skip lesions, giving a cobblestone appearance.

Answer 121

Skin - Perianal/mouth ulcers - Erythema nodosum - Pyoderma gangrenosum MSK - Arthritis - Seronegative spondyloarthropathy - Clubbing Eyes: - Conjunctivitis - Iritis

Answer 122

Faecal calprotectin raised Serology - pANCA Negative (more in UC) - ASCA positive (more in Crohns) Endoscopy + Biopsy - Endoscopy: Skip lesions, cobblestoning, strictures - Biopsy: Transmural inflammation, non caseating granulomas, goblet cells present Small bowel enema - High sensitivity/specificity for examination of terminal ileum - Stricturs (Kantor's string sign) - Proximal bowel dilation - Rose thorn ulcers - Fistulae

Answer 123

Stop smoking, NSAID Induce remission - Glucocorticoids: Budesonide (mild), prednisolone (moderate), IV hydrocortisone (very severe) - Immunosuppressant: Azathioprine or methotrexate - AntiTNF (infliximab) Maintain remission: - Azathioprine

Answer 124

80% of patients - Sticturing terminal ileus disease (ileocaecal resection) - Perianal fistulae (Diagnosed with MRI, treated surgically and draining seton left in to prevent abscess formation) - Perianal abscess (Surgical incision and drainage)

Answer 125

Small bowel cancer Colorectal cancer Osteoporosis Perianal fissure/fistula/abscess Anaemia/malnutrition

Answer 126

Relapsing and remitting bowel disease that usually involves rectum and can extend up large bowel, up to ileocaecal valve (does not affect anus). 3X more common in non smokers (smoking protective)

Answer 127

Continuous inflammation affecting only the mucosal layers. Usually starts at rectum, working its way proximally, never past the ileocaecal valve. Relapsing remitting course (flares with new damage, followed by healing) Regenerating mucosa forms a scar that looks like polyps (pseudopolyps)

Answer 128

- LLQ and tenesmus - Bloody, mucusy diarrhoea - Abdominal pain, urgency, weight loss and malnutrition Extraintestinal manifestations - Uveitis - Colorectal cancer - Erythema nodosum - Pyoderma gangrenosum - Arthritis - PSC!

Answer 129

Colonoscopy - Avoid colonoscopy if severe (perforation risk) - Red, raw mucosa - Continuous inflammation - Loss of haustrations Biopsy - Pseudopolyps, crypt abscesses, goblet cell depletion, inflammation limited to mucosal layers

Answer 130

Arthritis PSC and uveitis more common in UC

Answer 131

Mild <4 stools/day, small amount of blood Moderate 4-6 stools/day, varying amounts of blood Severe 6+ bloody stools/day

Answer 132

Induce remission Rectal (topical) aminosalicylate - mesalazine/sulfasalazine. Add high dose oral aminosalicylate, and then oral corticosteroid, if extensive or if remission not achieved in 4 weeks. Maintain remission Aminosalicylate + azathioprine. If severe/non responsive, Colectomy is curative

Answer 133

PSC Bowel perforation Toxic megacolon Colonic adenocarcinoma Strictures and bowel obstruction

Answer 134

Sudden, acute, severe UC flareup >10 bowel movements Continuous bleeding Abdominal tenderness Toxicity Colonic dilation Managed in hospital - IV corticosteroid - IV ciclosporin or infliximab Consider colectomy

Answer 135

- Infrequent voiding - Hurried micturition - Obstruction by full rectum (constipation) - Poor hygiene - Neuropathic bladder - Vesicoureteric reflux - developmental anomaly found in 35% UTI cases

Answer 136

Babies/infants non specific - Poor feeding, irritability, pain, fever, vomiting, urinary frequency Older children - Fever, suprapubic pain, vomiting, dysuria, urinary frequency, incontinence, haematuria - Temp >38 and loin pain suggest upper UTI (pyelonephritis) Suspect in kids with unexplained fever, vomiting, abdo pain and/or poor feeding

Answer 137

Urine dip - Clean catch best method, but urine collection pads can be used. Avoid other methods. Positive - leukocytes and nitrites. MSU Culture

Answer 138

- <3 months, refer to paeds - >3 months with upper UTI, admit, 7-10 days of antibiotics - >3 months lower UTI, 3 days trimethoprim, nitrofurantoin, cefalexin or amoxicillin

Answer 139

Recurrent - abdominal USS <6 weeks Atypical - Abdo USS during illness Should also be done for children under 6 months

Answer 140

Abnormal backflow of urine from bladder into ureter and kidney. Relatively common abnormality of the urinary tract which predisposes to UTIs. Ureters are displaced laterally, entering at a more perpendicular fashion.

Answer 141

Micturating cytourethorgram May use a DMSA (Dimercaptosuccinic Acid) scan to assess for damage, check for scarring.

Answer 142

Atypical or recurrent UTIs in kids under 6 months Family history Dilatation of the ureter on US VUR

Answer 143

1 - Reflux into ureter, no dilation 2 - Reflux into renal pelvis, no dilation 3 - Mild/moderate dilation of the ureter, renal pelvis and calyces 4 - Dilation of the renal pelvis and calyces and moderate ureteral tortuosity 5 - Gross dilation and tortuosity

Answer 144

Thrombosis in the small vessels around the body, usually triggered by Shiga toxins, either from E coli 0157 or Shigella. Abx used to treat these also increase risk Usually follows a gastroenteritis bout and has the classic triad: - Microangiopathic haemolytic anaemia (destruction of RBC due to thromboses in small vessels causing shearing) - AKI - Thrombocytopenia (low platelets)

Answer 145

Diarrhoea is first symptom, turns bloody in 3 days. One week after onset, HUS symptoms start - Fever - Abdo pain - Lethargy and pallor - Bruising - Haematuria - Jaundice - Confusion - Reduced urine output

Answer 146

Supportive treatment - Hypovolaemia (IV fluids) - Hypertension - Severe anaemia and renal failure (blood transfusion and haemodialysis)

Answer 147

Commoner in males as testis migrate from posterior abdominal wall through inguinal canal, but can occur in females if a patent processus vaginalis. Children presenting in first few months of life at risk of strangulation Treated with herniotomy without implantation of mesh.

Answer 148

Relatively common and may be found on newborn exam. Resolve on their own by 3 years

Answer 149

Incarceration - Cannot be reduced back into position Obstruction - Hernia causes a blockage Strangulation - Cuts off blood supply causing ischaemia. Surgical emergency.

Answer 150

Severe malnutrition caused by an overall deficiency in caloric intake (protein and energy). Usually kids under 5, in developing countries. Results in extreme fat and muscle loss, causing stunted growth, severe weight loss and immunodeficiency. Common in areas of famine or severe malnutrition

Answer 151

Rehydration Gradual intro of calories Micronutrient supplementation Long term nutritional rehabilitation and infection management

Answer 152

Severe protein malnutrition that occurs despite sufficient caloric intake. Often areas where diet is primarily starchy or carbohydrate heavy with little protein. Children often appear puffy due to generalised oedema (hypoalbuminaemia), especially in legs and face.

Answer 153

Sudden breastfeeding cessation Poverty Insufficient protein, high carbohydrate Food insecurity/scarcity

Answer 154

High mortality if untreated Heart failure (due to severe oedema) Sepsis Severe diarrhoea Refeeding syndrome if not treated properly

Answer 155

Electrolyte and fluid imbalance correction (slow, prevent osmotic demyelination syndrome) Gradual nutritional rehabilitation, protein rich etc

Answer 156

Inflammation of liver of newborns usually in first months of life. Can be due to viral infection, genetic disorders, or may be idiopathic. Inflammation leads to impaired bile flow, damaging liver

Answer 157

Viral (cytomegalovirus, hepatitis B) Metabolic disease (galactosemia, tyrosinaemia) Genetic disorders (alpha 1 antitrypsin deficiency) Family history and prematurity are RF

Answer 158

- Jaundice >first 2 weeks, prolonged - Hepatomegaly - Dark urine and pale stools - Poor feeding, failure to thrive

Answer 159

Majority of children are day and night continent by 3/4. If there is an involuntary discharge of urine by day or night, in a child aged 5 years or older, absent of congenital or acquired defects of the nervous system or urinary tract, this is enuresis. Primary - Never achieved continence Secondary - Child has been dry for at least 6 months

Answer 160

- 2 week fluid intake, toileting diary - Try to establish underlying cause Management Lifestyle: - Reduced fluid intake in evenings - Pass urine before bed - Positive reinforcement - avoid blame/shame/punishment (star chart for positive behaviours, e.g. going to the toilet before bed) - Enuresis alarms (requires long term training (>3 months) some may find this a burden/frustrating)

Answer 161

Daytime wetting, dry at night. More frequent in girls. - Urge incontinence: overactive bladder, little warning before leak - Stress incontinence: leakage during physical exertion, cough, laugh Other causes; - UTI - Psychosocial - Constipation

Answer 162

Desmopressin - Vasopressin analogue (anti-diuretic). Reduces volume of urine produced by kidneys, taken at night, for nocturnal enuresis. Can be used shorter term Oxybutinin - Anticholinergic, reduces contractility of bladder. Helpful in overactive bladder case Imipramine - Tricyclic antidepressant. Helps somehow

Answer 163

The inability for the foreskin to be retracted over the glans. Can be physiological or pathological (caused by scarring or inflammation) Physiological: Normal, 90% resolve by age 3. Pathological: Much less common, but caused by infection, scarring, trauma (forceful retraction), chronic inflammation/infections. Fibrosis of the preputial ring prevents retraction. Recurrent infection/inflammation may arise from poor hygiene

Answer 164

urinalysis, swabs, clinical examination. Treatment - Expectant if under 2 - Topical steroids - Circumcision or preputioplasty if severe or recurrent - Good hygiene!

Answer 165

Recurrent balanitis Urinary retention

Answer 166

A condition where the Urethral meatus (opening) is placed on ventral (under) side of the penis, towards scrotum. Also epispadias, where the meatus is displaced to dorsal (top) side of the penis Significant risk factor: Family history

Answer 167

- Ventral urethral meatus - Hooded prepuce - Chordee (head of penis bends down - ventrally) - Urethral meatus may open more proximally (Can be subcoronal, midshaft or penoscrotal)

Answer 168

May not need treatment if very distal. Surgery normally after 4 months (usually 12 months of age). Essential child NOT circumcised before surgery, as foreskin can be used in procedure. Surgery aims to correct meatus position and straighten penis

Answer 169

Wilms' nephroblastoma, one of the most common childhood malignancies (typically presents under 5). Kidney tumour

Answer 170

Child under 5 presenting with abdomen mass. - Painless haematuria - Flank pain - Unilateral - HTN - Lethargy, weight loss - 20% metastasise to lung

Answer 171

- Unexplained enlarged abdo mass = arrange paediatric review within 48 hours - USS to visualise kidneys, CT/MRI for staging, biopsy for definitive diagnosis - Managed with nephrectomy of affected kidney - Chemo and radio may be needed - Prognosis good - 80% cure

Answer 172

Inflammation of nephrons of kidneys. Presents with: - Haematuria - Oliguria (low urine) - Hypertension (Na+ retention) - Oedema - Slight proteinuria BUT <3.5g of protein in 24hrs (Anymore = nephrotic syndrome) Most common causes of nephritis in kids are post-strep glomerulonephritis and IgA nephropathy

Answer 173

Kidney inflammation that occurs 1-3 weeks after a B haemolytic strep infection (e.g. tonsilitis caused by Strep pyogenes, or skin infection). IgG/IgM/C3 Immune complexes deposit in glomerular basement membrane causing activation of complement and inflammation Type 3 hypersensitivity. Strep pyogenes is beta haemolytic, group A

Answer 174

Recent (1-3 weeks) tonsilitis, or skin infection (6 weeks) Proteinura and microscopic Haematuria Oliguria Peripheral oedema Hypertension Signs of recent infection/sore throat history!

Answer 175

Throat swab & culture - Strep pyogenes Anti-streptolysin O titre - increased Low C3 Renal biopsy results: - Acute, diffuse, proliferative glomerulonephritis - Endothelial proliferation with neutrophils - Immunofluorescence: "Starry sky" appearance caused by IgG, IgM, C3 deposits - Electron microscopy: subepithelial humps caused by lumpy immune complex deposits

Answer 176

PSG - 1-2 weeks post URTI - Proteinuria>haematuria (which is microscopic) - Low complement IgA - 1-2 days post URTI - Young males - Macroscopic haematuria

Answer 177

No specific (Penicillin for underlying strep, Furosemide for oedema/HTN, use ACEi if very severe) - Pulmonary oedema - Hypertensive encephalopathy - Severe AKI

Answer 178

AKA Berger's disease. IgA deposition in mesangium of kidney (Bowman's capsule) Commonest cause of glomerulonephritis worldwide. Presents as macroscopic haematuria in young people (teenagers, YA). Associated with Henoch-Schonlein Purpura (IgA vasculitis), coeliac, alcoholic cirrhosis Type 3 hypersensitivity (antigen-antibody complex deposition)

Answer 179

Young male, recurrent macroscopic haematuria, starting 1-2 days post recent URTI.

Answer 180

Isolated haematuria, or minimal proteinuria <1000mg/day - no treatment, just follow up. ACE inhibitors main treatment Corticosteroids if failure to respond or low eGFR.

Answer 181

Urinalysis - Macroscopic haematuria C3/C4 - NORMAL Renal biopsy - Immunoflourescence: IgA and C3 - Histology: Mesangial proliferation, immune complexes

Answer 182

Worse than post-strep 25% develop end stage renal failure High proteinuria (>2g/day), hypertension, smoking mark poor prognosis

Answer 183

Type 2 hypersensitivity AKA Anti-GBM (Glomerular Basement membrane) disease, autoantibodies attack type 4 collagen in glomerular and alveolar membrane causing haematuria and haemoptysis (bloody cough first) Anti-GBM and p-ANCA positive Treated with corticosteroids

Answer 184

Glomerular BM is damaged and becomes permeable to protein. Triad - Proteinuria (>3.5g/24hrs) - Hypoalbuminaemia (lost in urine) - Peripheral oedema (loss of oncotic pressure)

Answer 185

Hyperlipidaemia (causing frothy urine) Hypercoagulability (frothy urine) Hypertension Susceptibility to infection (Loss of Ig in urine)

Answer 186

Most common cause in children: Minimal change disease. Also due to intrinsic kidney disease: - Focal segmental glomerulosclerosis - Membranoproliferative glomerulonephropathy

Answer 187

Most common nephrotic in children, usually presents before 8. Aetiology not usually clear. Associated with Hodgkins lymphoma, leukaemia and NSAID use. Causes oedema, proteinuria and low albumin, in a usually otherwise well child.

Answer 188

Urinalysis - Small molecular weight proteins and hyaline casts Renal biopsy - Light microscopy - normal - Electron microscopy - fusion of podocytes and effacement of foot processes

Answer 189

Oral corticosteroids 1/3 have just 1 episode 1/3 have infrequent relapse 1/3 have frequent relapse which stop before adulthood.

Answer 190

Female Structural urological abnormalities Vesico-ureteric reflux Diabetes

Answer 191

Inflammation of kidney from bacterial infection. Affects renal pelvis and parenchyma. Usually ascending. Main cause is E Coli (gram negative, anaerobic rod) Klebsiella Enterococcus E coli Pseudomonas Staph aureus

Answer 192

Triad - Fever - Loin/back pain - Pyuria (WBC/pus in urine) Otherwise; - Dysuria - Frequency - Suprapubic discomfort - Systemic illness - Renal angle tenderness on exam - Haematuria

Answer 193

Examination - Tender loin - Renal angle tenderness Urine dip - nitrites, leukocytes, blood Midstream urine MC+S CT for imaging

Answer 194

IV fluids and broad spectrum antibiotics (Co-Amox 14 days) Drain obstructed kidney and remove catheter

Answer 195

Renal abscess Progression to chronic - which can progress to end stage renal failure. DMSA Scan used in recurrent pyelonephritis to assess for renal damage.

Answer 196

Atopic condition (Atopic dermatitis) causing inflammation of skin. Occurs mostly over flexor surfaces (Inside of elbows and knees) but also: - Face - Neck - Trunk - (Face and neck in infants, flexor surfaces and creases of face and neck mostly in older kids)

Answer 197

Flare treatment and maintenance Pharmacological: - Simple emollients to start if mild - Topical corticosteroids (hydrocortisone mild, clobetasone butyrate if moderate etc.) - Topical calcineurin inhibitors (tacrolimus) - Oral antihistamine Non pharm: - Avoid triggers - Wet wrap therapy for severe or chronic flares

Answer 198

Viral skin infection in patients with eczema caused by herpes simplex virus 1 (HSV-1) or Varicella Zoster virus (VZV). Can be associated with a coldsore in the patient or close contact. Causes widespread, painful, vesicular rash with systemic symptoms (fever, lethargy, irritability, reduced oral intake and lymphadenopathy). Leaks pus and leave punched out ulcers with red base after vescicles burst.

Answer 199

Viral swabs used to confirm, but treat first. Treated with oral aciclovir unless severe (then IV). Can be life threatening if immunocompromised. Bacterial superinfection can occur

Answer 200

Chronic autoimmune recurrent psoriatic skin lesions. Genetic association. Worsened by stress, environmental factors, irritation, infection, etc. Sunlight generally improves symptoms

Answer 201

Plaque: Most common, well-demarcated red, scaly patches on extensor surfaces, sacrum and scalp. Guttate: Most common in kids. Small raised papules on trunk and limbs. Can turn into plaques. "Teardrop lesions". Often triggered by Strep throat infection, often resolves within 3-4 months Pustular: Rare, pustules under erythematous skin, leaks non infective pus, occurs on palms and soles. Treated as medical emergency

Answer 202

Plaque most common, Guttate most in kids, often triggered by throat infection. Dry, flaky, scaly, faintly erythematous skin lesions, that appear in raised and rough plaques. Most commonly in extensor surfaces of elbows and knees and on scalp. Signs suggesting it: - Auspitz: small points of bleeding, when plaques scraped off - Koebner: psoriatic lesions to areas of skin affected by trauma - Residual pigmentation: After lesions resolve.

Answer 203

Causes psoriatic arthritis with nail pitting, discolouration, thickening and onycholysis (seperation from nail bed). Subungal hyperkeratosis and loss of nail also. Psoratic arthritis in 10-20%.

Answer 204

Regular emollients. Stepwise approach: - 1: Potent corticosteroid + vitamin D analogue (applied seperately! - Day/Night), for up to 4 weeks. - 2: Vit D analogue twice daily - 3: Potent corticosteroid twice daily OR Coal tar preparation 1/2 times a day.

Answer 205

Phototherapy: - Narrowband UV B light 3 times a week - Adverse effects: Skin ageing, squamous cell cancer Systemic: - Oral methotrexate if joint disease. - Ciclosporin

Answer 206

Potent topical corticosteroids once daily for 4 weeks. Shampoo or mousse can be used, with/without a topical agent before application of the corticosteroids

Answer 207

- Mild/moderate potency corticosteroid 1/2 times a day for 2 weeks

Answer 208

- Topical steroids can cause skin atrophy, striae, rebound symptoms, hence using on flexural surfaces less. - Topical used over large area can cause systemic symptoms - 4 week break before next round of topical steroids - No longer than 8 weeks, potent steroids for no less than 4

Answer 209

Calcipotriol (dovonex), calcitriol etc Decrease cell division and differentiation, reducing epidermal proliferation. Can be used long term, and do not stain or smell like coal. Reduce scale and plaque thickness but not redness. Avoid in pregnancy! (risk of hypercalcaemia, or calcium deposition in fetal kidneys)

Answer 210

Inflammation and obstruction of the pilosebaceous follicle with keratin plugs which results in comedones, inflammation and pustules. Sebaceous glands produce sebum, increased production traps keratin. Comodones can be open (blackheads) or closed Associated with colonisation of anaerobic bacterium Propionibacterium acnes Typically affects the face, neck and upper trunk.

Answer 211

Comedones - Dilated sebaceous follicle, white if closed, black if open Inflammatory lesions when follicle bursts, causing papules (small lumps) and pustules (small lumps with yellow pus) Inflammation may be excessive causing nodules and cysts Scarring can be: - Hypertrophic (lumps) - Ice-pick (indentations) - Hyperpigmentation (darkening of skin)

Answer 212

Drug Induced - monomorphic Fulminans - Includes systemic upset, requires hospital admission and responds to oral steroids

Answer 213

Mild: Open and closed comedones, may have sparse inflammation Moderate: Widespread non inflammatory lesions and nuerous papules and macules Severe: Extensive inflammation, nodules, pitting, scarring.

Answer 214

Mild - 12 week topical combination therapy - Topical adapalene and topical benzoyl peroxide - Topical tretinoin and topical clindamycin - Topical benzoyl peroxide and topical clindamycin Moderate/severe - 12 week course of Topical adapalene and benzoyl peroxide with oral lymecycline or doxycycline

Answer 215

- Tetracyclines contraindicated in women breastfeeding, pregnant or in children under 12. Erythromycin can be used - Topical retinoid or oral benzoyl peroxide should be co prescribed with oral abx to prevent resistance - Using antibiotics for >6 months may cause gram negative folliculitis, treat with oral trimethoprim

Answer 216

monotherapy with topical or oral antibiotic, or just both together. Due to risk of resistance

Answer 217

Oral retinoids (TERATOGENIC) COCP in women Co-cyprindiol (most effective COCP but has higher risk of VTE) Isotretinoin - reduces sebum production. Strongly teratogenic, must be on reliable contraception and stop for a month before pregnancy

Answer 218

Stevens Johnsons and Toxic Epidermal Necrolysis are the same thing, just on less than 10% and more than 10% of the body respectively. A disproportional immune response causes epidermal necrosis

Answer 219

Medications - Anti-epileptics (lamotrigine, carbamazepine,phenytoin) - Penicillin - NSAID - Allopurinol Infections - HSV - Mycoplasma Pneumonia - Cytomegalovirus - HIV Certain HLA

Answer 220

Starts non specific (fever, cough, sore throat, itchy skin) followed by purple rash that spreads and blisters. Skin starts to break away and shed, leaving raw tissue underneath. Can affect mucuous membranes of mouth and eyes, causing inflammation, blistering and shedding. Rash is maculopapular with target lesions, that can turn into vescicles or bullae. - Nikolsky sign positive in erythematous areas (blisters and erosions appear with gentle rubbing) - Mucosal involvement and systemic symptoms

Answer 221

Medical emergency, immediate admission. Treated with steroids, immunoglobulins and immunosuppression

Answer 222

Type 1 hypersensitivity (IgE mediated) Nose becomes sensitised to triggers such as dust mites, grass, tree, pollens, pets, mould. Can be: - Seasonal - Perennial (year round) - Occupational

Answer 223

Sneezing Bilateral clear nasal discharge Post nasal drip and itching Itchy red swollen eyes

Answer 224

Pale, pink, raised skin that are itchy. Allergic response. Mast cell and histamine mediated. Can be acute (allergic, virus, bites, contact) or chronic - Chronic idiopathic - no known cause - Chronic inducible (sunlight, temperature, exercise, stress) - Autoimmune (associated with autoimmune disease - antibodies target mast cells) Managed with antihistamines, e.g. Fexofenadine. Sedating antihistamine (chlorphenamine) can be used at night Short course of oral can be used

Answer 225

Penicillin Aspirin NSAID Opiates

Answer 226

Severe allergic response characterised by sudden and rapidly progression Airway Breathing and Circulation symptoms Airway - Swelling of throat and tongue, causing hoarse voice and stridor Breathing - Wheeze and dyspnoea Circulation - Hypotension, tachycardia Also causes - Urticaria/angioedema - Itching - Collapse

Answer 227

IMMEDIATE IM adrenaline 500mcg on the anterolateral aspect of the thigh (>12yo). Can be repeated every 5 mins if necessary. A - Secure airway B - Provide oxygen/salbutamol C - IV fluid bolus D - Lie flat to improve cerebral perfusion E - Look for flushing, urticaria, angioedema

Answer 228

<6 months 100-150mcg 6 months-6 years - 150mcg 6-12 years - 300mcg 12+ - 500mcg

Answer 229

All children should be monitored, in case of biphasic reaction. Serum mast cell tryptase should be measured to confirm diagnosis within 6 hours. Provide adrenaline auto injector, with BLS training.

Answer 230

Irritant dermatitis - Most common cause, due to urinary ammonia and faceces, does not affect creases Candida dermatitis - Erythematous rash which involves flexures and has satellite lesions Seb Dermatitis - Erythematous rash with flakes, that may also affect scalp Psoriasis - Red scaly rash that may be present elsewhere

Answer 231

Disposable nappies Expose to air Apply barrier cream or steroid cream Candidal nappy rash treated with topical imidazole

Answer 232

It is a systemic, medium sized vessel vasculitis. Typically under 5. Big complication is a coronary artery aneurysm

Answer 233

Persistent high fever (>39C) for more than 5 days. Key findings: - Widespread erythematous maculopapular rash and desquamation (skin peeling) on palms and soles - Strawberry tongue - Red, cracked lips - Cervical lymphadenopathy - Bilateral conjunctivitis

Answer 234

Acute: Child most unwell with fever, rash and lymphadenopathy 1-2 weeks Subacute: acute settle, desquamation and athralgia occurr and risk of coronary artery aneurysms. Lasts 2-4 weeks. Convalescent stage: Symptoms settle and bloods return to normal. 2-4 weeks

Answer 235

High dose aspirin to reduce thrombosis risk IV immunoglobulins to reduce risk of coronary artery aneurysm

Answer 236

Reyes syndrome risk

Answer 237

RNA paramyxovirus, spread by aerosol transmission. Has a 10-14 day incubation period and patient is infective from prodrome until 4 days after rash start. MMR Vaccine vaccinates against it

Answer 238

Prodrome - Irritable, conjunctivitis, fever Followed by main features - Koplik spots (white spots (grains of salt) on the buccal mucosa) appear before the rash - Rash (Starts behind ears (3-5 days post fever) and spreads to whole body. Discrete maculopapular rash that becomes blotchy and confluent. Desquamation typically spares palms and soles)

Answer 239

Measles is self resolving 7-10 days post symptoms. Isolate until 4 days post symptom resolution Notifiable disease to UKHSA Vaccinate contacts within 72 hours

Answer 240

Otitis media - most common Pneumonia - most common cause of death Encephalitis Diarrhoea Meningitis Hearing loss Vision loss Death

Answer 241

Erythrogenic toxins produced by Group A haemolytic streptococci (Strep pyogenes), more common in kids 2-6 years, peak at 4. Presents as widespread rough "sandpaper" rash following a Group A Strep infection (e.g. tonsilitis). Spread via respiratory droplets

Answer 242

2-4 day incubation period and presents with: - Fever 24-48 hours - Strawberry tongue - Rash (fine punctuate erythema "pinhead" which appears first on torso and spreads, sparing palms and soles. Red-pink, blotchy, macular rash with rough "sandpaper" skin. Desquamination occurs) - Cervical lymphadenopathy - Flushed face, sore throat, malaise.

Answer 243

- Throat swab to confirm - Oral Penicillin V for 10 days - Azithromycin alternative - Notifiable disease - Can return 24 hours after commencing antibiotics

Answer 244

Usually mild but: - Otitis Media (most common) - Rheumatic fever - Post streptococcus glomerulonephritis are possible

Answer 245

Rubella virus (part of togavirus family), highly contagious and spread via respiratory droplets. 2 week incubation. Infective from 7 days before symptoms to 4 days after rash Presents with erythematous macular rash and is notifiable. Children stay off school 5 days, avoid pregnant women

Answer 246

Prodrome: low grade fever Rash: Erythematous maculopapular rash initially on face before spreading to rest of body. Lymphadenopathy: Suboccipital and postauricular

Answer 247

Supportive. Notify UKHSA Children stay off school and avoid pregnant women

Answer 248

Congenital rubella syndrome Damage most likely in 8-10 weeks - Senisorineural deafness - Congenital cataracts - Congenital heart defects - Cerebral palsy - Hepatosplenomegaly - Microphthalmia (Deafness, blindness and congenital heart disease is a triad for this condition)

Answer 249

- IgM antibodies raised in mother. - Hard to distinguish from parovirus B19 clinically, so worth checking both. Management - Inform local health protection unit - Advise non immunised to avoid rubella contacts,

Answer 250

DNA virus which causes a range of clinical presentations. Can go anywhere from mild fever to full rash. Rash presents bright red on both cheeks (AKA Slapped cheek syndrome) before reticular rash appears over trunk and limbs. (rarely palms/soles) As rash appears, child is no longer infectious.

Answer 251

Warmth (warm bath, heat, fever) can trigger rash again, but treatment and school exclusion are unnecessary

Answer 252

- Miscarriage/fetal death - Severe fetal anaemia - Hydrops fetalis - Maternal pre-eclampsia like syndrome (hydrops fetalis, placental oedema, oedema in mother) IgM to parvovirus (acute infection, last 4 weeks) IgG parvovirus long term immunity Rubella antibodies (Important differential!)

Answer 253

Caused by HHV-6 and HHV-7 (human herpes virus) High fever for 3-5 days, then disappears suddenly. Rash then appears for 1-2 days. Mild erythematous macular rash across legs, arms, trunk and face, not itchy. Main complication is febrile convultions due to high temperature If immunocompromised, GBS, myocarditis, thrombocytopaenia.

Answer 254

Zika virus, spread by Aedes mosquito. Can be spread sexually. Congenital Zika - Microcephaly - Fetal growth restriction - Vetriculomegaly, cerebellar atrophy.

Answer 255

- Primary: Chicken pox, indicated in healthcare workers and contacts of immunocompromised patients. - Reactivation in dorsal root ganglion (usually at later age): shingles, indicated in those 70-79 Both are live attenuated vaccines

Answer 256

- Initial fever - Itchy rash that starts on face/trunk, then spreads. Starts as small red macules, that rapidly progress to vescicles (blisters) that rupture and crust over, forming scabs. Happens over 5 days. Most infectious 1-2 days before rash, and infective until all scabs have crusted over. - Mild systemic symptoms (malaise, fatigue)

Answer 257

Bacterial infection of the lesions - can be small cellulitis or necrotising fasciitis caused by invasive group A streptococcal infection. NSAIDs increase risk of NF!! Rare - Pneumonia - Encephalitis - Shingles/Ramsay Hunt syndrome (reactivation of virus)

Answer 258

Calcific miliary opacities. Dense, with no uniform size.

Answer 259

<20 weeks. Causes congenital varicella syndrome (developmental problems) Treated with Varciella Zoster immunoglobulins. If infection around time of delivery, use IVIG and aciclovir

Answer 260

Fetal varicella syndrome (+5x risk of pneumonitis to mother) - Risk is 1% <20 weeks g, unlikely >28 weeks - Causes skin scarring, microphthalmia, limb hypoplasia, microcephaly, learning disabilities Other risks include - Shingles in infancy (dorsal root ganglion reactivation) - Severe neonatal varicella

Answer 261

Oral aciclovir between day 7-14 post exposure.

Answer 262

Infection that causes fever, sore throat with grey pseudomembrane and lymphadenopathy. Vaccinated against in 6-in-1 vaccine (toxin vaccine). Caused by Cornyebacterium diphtheriae which releases an exotoxin encoded by B-prophage, which inhibits protein synthesis by catalysing ADP-ribosylation of elongation factor 2 (EF2)

Answer 263

Recent visitor to Eastern Europe/Russia/Asia - Sore throat with "diphtheric membrane" - grey, pseudomembrane on posterior pharyngeal wall. - Bulky cervical lymphadenopathy - Neuritis - Can cause heart block Diphtheric membrane on tonsils caused by necrotic mucosal cells. Systemic distribution can cause necrosis of myocardial, neural and renal tissue, causing heart block, neuritis, peripheral neuropathy (motor loss) etc

Answer 264

- Throat swab and culture - using tellurite agar or Loeffler's media - Managed with IM penicillin or diphtheria antitoxin

Answer 265

A skin infection caused by Staph aureus in which exfoliative toxins (ETA and ETB) cause widespread erythema and blistering, which burst, causing desquamation that resemble burns. The lesions do not affect mucosal surfaces, distinguishing it from TEN.

Answer 266

Usually under 5 - May be preceded by fever, irritability, malaise - Widespread erythema followed by large, flaccid blisters that easily rupture. These then peel (desquamation), resembling a scald. - Mucosal surfaces are UNAFFECTED

Answer 267

Managed with wound care (cleaning) supportive (hydration/fluid balance), and antibiotics (fluclox or vancomycin if MRSA) - Secondary infection (cellulitis/pneumonia) or dehydration and electrolyte imbalance are main concerns.

Answer 268

Gram negative bacterial infection Bortadella pertussis, typically presenting in children. Sometimes called 100 day cough Notifiable disease. Child has severe coughing fits in which child struggles to take air between coughs. Vaccinated against but vaccine nor immunisation provide lifelong protection

Answer 269

Catarrhal phase - 2 weeks (extremely contagious) - low grade symptoms, fever, rhinorrhoea, cough. Paroxysmal phase 1-6 weeks - Uninterrupted “whooping” coughing that can result in vomiting, collapsed lung, broken ribs and apnoea - O2 decrease can cause death - Inspiratory whoop - Usually worse at night or after feeding Convalescent phase (healing) - Cough subsides over weeks/months

Answer 270

Unexplained acute cough >14 days + at least: - Paroxysmal cough (Paroxysmal = in attacks/episodes) - Inspiratory whoop - Post-tussive vomiting - Undiagnosed apnoeic attacks in children Investigated for using - Nasal swab culture for Bodetella pertussis - PCR and serology

Answer 271

Notifiable, notify UKHSA Oral macrolide (azithromycin, clarithromycin etc) if cough started within 21 days. Oral prophylaxis for household contacts School exclusion until 48 hours after starting antibiotics.

Answer 272

Symptoms usually resolve in 8 weeks but severe cough can cause damage: Bronchiectasis! Pneumonia Rib fractures Encephalopathy/Seziures

Answer 273

Poliomyelitis, caused by poliovirus. Spread through faeco-oral and respiratory routes. Mainly affects kids' nervous systems, can lead to paralysis. Attacks the anterior horn cells of the spinal cord, causing flaccid paralysis. Usually vaccinated in 6in1 (inactivated vaccine), so rare here but endemic in afghanistan, pakistan and nigeria.

Answer 274

Respiratory failure Long term post polio syndrome, causing muscle weakness and fatigue for decades Permanent paralysis

Answer 275

Inflammation of the leptomeninges due to an infection of the meninges and CSF. Usually due to N Meningitidis (gram negative diplococci) or Strep pnuemoniae Notifiable disease

Answer 276

Neonatal (<3 months) - Group B strep (acquired from mother at birth - more common in low birth weight or prolonged ROM) - E coli - Listeria monocytogenes (avoid cheese) <6 years - N mengitidis - S pneumoniae - H influenza > 6 years - N meningitidis - S pneumoniae

Answer 277

Group B strep lives harmlessly on maternal vagina

Answer 278

- N meningitidis - Gram negative diplococci (Only one that causes non blanching rash!) - S pneumoniae/Group B strep - Gram positive cocci in chains - Listeria monocytogenes - Gram positive bacillus

Answer 279

Signs - Neck stiffness, headache, photophobia (avoids light) - Phonophobia (avoid sound) - Papilloedema (optic disk swelling) - Non blanching rash (N meningitidis only) Pyrexia, reduced GCS Kernig sign - Patient lie on back, flex leg and knee to 90. Straightening leg causes neck pain Brudzinski sign - Lie patient on back and passively flex neck to chest, this will cause involuntary hip and knee flexion

Answer 280

Can be non specific - Hypotonia - Poor feeding - Lethargy - Hypothermia - BULGING FONTANELLE Lumbar puncture if under 1 year with fever/unwellness

Answer 281

Meningococcal septicaemia

Answer 282

Blood culture 1st line Lumbar puncture GOLD Bacterial - Cloudy/yellow - High protein - Low glucose - High WCC (neutrophil) Viral - Clear appearance - Protein small raise/normal - Normal glucose - WCC high (Lymphocytes)

Answer 283

Meningococcal septicaemia (non blanching rash presence) Any sign of raised ICP - Focal neurological signs - Papilloedema - Significant bulging of the fontanelle - Disseminated intravascular coagulation - Cerebral herniation

Answer 284

<3 months - IV Cefotaxime and amoxicillin >3 months - IV Ceftriaxone + Dexamethasone ONLY if > 3 months and bacterial Prophylaxis of contacts: - Ciprofloxacin (Contacts within 7 days prior to onset at greatest risk)

Answer 285

Primary care - Immediate IM Benzylpenicillin if meningococcal suspected Hospital - Cefotaxime or Ceftriaxone + Dexamethasone - Add amoxicllin if <3 or >50 to cover Listeria - Contact tracing and Ciprofloxacin for contacts

Answer 286

- Hearing loss! (sensorineural) - Cerebral palsy - Seizures, memory loss, cognitive impairment - Infection can cause sepsis and intracerebral abscess - Pressure can cause brain herniation and hydrocephalus Meningococcal can lead to Waterhouse-Friedrichsen (adrenal insufficiency secondary to adrenal haemorrhage)

Answer 287

Inflammation of the brain, commonly due to viral cause. Common cause in children is HSV-1 from cold sores but can also be due to HSV-2 from genital herpes, contracted at birth (common in neonates) Herpes simplex encephalitis characteristically affects temporal lobes

Answer 288

VZV (Chickenpox) Cytomegalovirus (immunodeficiency) EBV (infectious mononucleosis, enterovirus, adenovirus, influenza)

Answer 289

- LP: CSF clear with lymphocytosis and elevated protein - PCR for HSV - CT: Medial temporal and inferior lateral changes. - MRI preferred imaging EEG shows localised discharges at 2Hz

Answer 290

IV Aciclovir

Answer 291

Superficial skin infection usually caused by Staph aureus or Strep pyogenes. Contagious, and children should be kept off school during disease course. Can be primary or secondary to eczema, scabies, insect bites Gold crust characteristic of Staph infection

Answer 292

Can be bullous or non bullous - bullous is always staph aureus. Non bullous - Typically around nose/mouth, where exudate from golden crust. Does not cause systemic symptoms Bullous - Staph aureus produce epidermolytic toxins that break down proteins that hold skin together, forming 1-2cm vescicles that burst forming a golden crust. These eventually heal without scarring. Bullous more common in neonates and children. When severe and widespread, it is called staphylococcal scalded skin syndrome.

Answer 293

- "Golden Crust" from skin lesions typically found around mouth - Very contagious - stay off school! - Spread through itching of scabs

Answer 294

Non-bullous/not systemically unwell - hydrogen peroxide 1% cream Bullous - Topical fusidic acid - Topical mupriocin if fusidic resistant Extensive disease - Oral flucloxacillin Keep off school until lesions are crusted/healed or 48 hours post starting Abx

Answer 295

Usually responds well to treatment but if not; - Cellulitis - Sepsis - Scarring - Post strep glomerulonephritis - Staph scalded skin syndrome - Scarlet fever

Answer 296

Caused by a severe systemic reaction to staphylococcal exotoxin TSST-1 superantigen toxin. Previously caused by infected tampons Presents with fever (>39C), hypotension, diffuse erythematous rash with desquamation, and involvement of 3 or more organ systems. Managed with IV fluids and antibiotics

Answer 297

- Unprotected sexual activity - Mother to child (breastfeeding, pregnancy, birth - can be any stage) - Mucous membrane, blood, open wound exposure to infected blood or body fluid. (Sharing needles, needle stick, blood splash in eye).

Answer 298

Delivery - Vaginal if viral load <50/ml - C Section if >50/ml - Give IV Zidovudine during C section if unknown or >10,000 Prophylaxis - Low risk (<50/ml in mother) zidovudine for 4 weeks - High risk (>50/ml) zidovudine, lamivudine and nevirapine 4 weeks Do not breastfeed, regardless of load

Answer 299

HIV antibody screen - Checks for antibodies against HIV. Can be false positive, due to antibodies crossing placenta. Can also take 3 months post exposure to develop antibodies. HIV viral load - Tests directly for viruses in blood. Test when - Babies to HIV positive parents - When immunodeficiency suspected (e.g. recurrent illness) - Risk factors such as IV drug use, needle stick or sexually active

Answer 300

Co-trimoxazole

Answer 301

Antiretroviral therapy - Normal CD4 count - Undetectable viral load

Answer 302

Vaccine that offers limited protection against TB. Given to high risk infants. Greenbook advises: - All infants in areas of UK with > incidence of TB than 40/100,000 - All infants with parent or grandparent from country with TB - Previously unvacinated, tuberculin negative contacts of TB patients. Contains live attenuated Mycobacterium bovis, also prevents leprosy. Must have tuberculin test first.

Answer 303

A type of seizure that occur in children with a high fever. Typically occur between 6 months and 5 years, not associated with pathology. Usually found in early viral illness, last less than 5 mins and mostly tonic-clonic

Answer 304

Simple - <15 mins, generalised, no reccurence within 24 hours (may be solitary), complete recovery within an hour. Complex - 15-30 mins, focal seizure, repeat within 24 hours Febrile status epilepticus - >30 mins

Answer 305

Diagnosis of exclusion. - Epilepsy - Meningitis - Space occupying lesions - Syncopal episode - Electrolyte abnormalities Typical presentation - 18 month child with 2-5 min tonic clonic seizure during a high fever. Due to underlying viral illness or bacterial such as tonsilitis. Source of infection should be investigated.

Answer 306

First seizure or complex - admit to paediatrics. Ongoing - can be managed at home, but call ambulance if >5 mins. - If recurrent, benzodiazepine (rectal diazepam or buccal midazolam) can be used.

Answer 307

1/3 will have further febrile convulsions. Increased risk of epilepsy (2.5% for no risk, much higher if all or complex convulsions) Risk factors for further seizures: - Age <18 months - Fever <39 - Shorter duration of fever before seizure - Family history

Answer 308

Infantile spasms (West Syndrome) - Brief spasms in the first few months of life - Key features: Flexion of head, trunk, limb and extension of arms (Salaams attacks), which last 1-2 seconds and can repeat up to 50 times a day. - Usually secondary to a serious neurological condition (tuberous sclerosis, encephalitis, birth asphyxia) - Vigabatrin and prednisolone are possible treatments. - Poor prognosis - 1/3 dead by 25

Answer 309

Possible extension of infantile spasms, affecting 1-5 yo. - Presents with atypical absences, falls and jerks. Atonic seizures - 90% of patients will have moderate-severe mental handicaps - Ketogenic diet may help

Answer 310

First line: Sodium valproate (only in males as teratogenic - neurodevelopmental delay) In females: Lamotrigine

Answer 311

Seizures affecting 1 area of the brain. Usually start in temporal lobe, affecting hearing, speech, memory and emotions. Simple: Remains conscious, no post ictal symptoms and memory of event Complex: LOC. Impaired memory, post ictal. Treated with: First line: carbamazepine or lamotrigine Second line: sodium valproate or levetiracetam (reverse of tonic-clonic!)

Answer 312

Temporal: Aura - Rising epigastric sensation. Followed by dejavu, hallucinations, automatisms (doing weird things on autopilot). Frontal (motor) - Head/leg movments, posturing, jacksonian march (start in certain muscle group and move). Post ictal weakness Parietal (sensory) - paraesthesia Occipital (visual) - Floaters/flashes etc

Answer 313

Typically in children. Goes blank, stares into space and abruptly back to normal. Last 10-20 seconds. Managed with Sodium valproate or ethosuximide

Answer 314

Atonic - drop attacks (lapses in muscle tone) - <3 mins - suggest lennox-gastaut syndrome Myoclonic - Brief muscle contractions (sudden jump). Patient usually awake. Happen as part of juvenile myoclonic epilepsy

Answer 315

Grand - Tonic clonic Petit - Typical absence

Answer 316

Investigate in kids after second simple seizure EEG and brain MRI MRI considered when: - First seizure under 2 years - Focal seizures - No response to medication Also do - ECG (exclude cardiac cause) - Electrolytes - Blood cultures, urine cultures and lumbar puncture to rule out sepsis, encephalitis, meningitis (prolactin is also raised immediately after a seizure for some reason)

Answer 317

First line in all except focal Works by increasing GABA activity, relaxing brain - Teratogenic - Liver damage - Hair loss - Tremors

Answer 318

Na+ channel blocker and suppresses glutamate release, reducing excitability of neurones - Blurred vision - Leukopenia - Stevens-Johnson syndrome (life threatening rash) or DRESS syndrome First line in women/pregnant

Answer 319

Sodium channel blocker, reduces excitability of neurones - Agranulocytosis - Aplastic anaemia - Dizziness - Rash

Answer 320

Night terrors Rash

Answer 321

A seizure >5 mins, or >2 seizures without regaining consciousness. Medical emergency - ABCDE approach - IV lorazepam in hospital or buccal midazolam or PR diazepam in community If ongoing - IV phenytoin or phenobarbital

Answer 322

Childhood epilepsy (4-12) At night/when waking up. Typically partial (e.g. paraesthesia affecting face) Centrotemporal spikes on EEG. Excellent prognosis

Answer 323

3Hz generalised, symmetrical spike on EEG

Answer 324

Granulomatous resp and non resp infection. Type 4 hypersensitivity. Notifiable Mycobacterium tuberculosis, acid fast bacilli. Slow dividing and have high oxygen requirements - difficult to culture. As it is acid fast, a Ziehl-Neelsen stain must be used, turning them bright red against a blue background. 1.7b cases worldwide - mostly latent in South Asia/Sub Saharan Africa

Answer 325

Primary - TB phagocytosed but resists killing (waxy mycolic capsule prevents binding - acid fast) Focal, caseating granuloma forms (Ghon focus). If it involves hilar lymph nodes, it becomes a Ghon complex Latent - Bacteria dormant, patient asymptomatic. Sputum test will be negative but Mantoux will still be positive Secondary - Immunocompromised patients, TB reactives and patient is symptomatic and infectious. Miliary - Lymphatogenous spread to other organs, causing systemic symptoms

Answer 326

Pott's Disease

Answer 327

TB meningitis Vertebral bodies (Pott's disease) Cervical lymph nodes (scrofuloderma) TB pericarditis Renal/GU tract

Answer 328

Chest X ray (signs on other card) Sputum culture - 3 deep cough sputum culures, which stain red with Ziehl-Neelsen stain. Nucleic acid Amplification Test - Done on urine or sputum if under 15 or has HIV.

Answer 329

Primary - Patchy consolidations - Pleural effusion - Hilar lymphadenopathy Latent - Ghon complex Secondary - Nodular consolidation with cavitation (gas filled spaces) in upper lung Miliary - "Millet Seeds" uniformly distributed throughout lung

Answer 330

Mantoux screening - Intradermal injection of purified protein derivative which causes a type 4 hypersensitivity reaction. After 72 hrs, >15 mm induration is strongly positive, and suggests infection. 6-15 may be due to BCG or due to previous TB. Interferon gamma release assay - Blood mixed with TB. If previous contact, will release interferon gamma.

Answer 331

Recent infection HIV Sarcoidosis Lymphoma

Answer 332

BCG Intradermal injection of live attenuated Mycobacterium bovis. Protects mostly against severe and complicated TB, but less against pulmonary TB.

Answer 333

RIPE - two months course Rifampicin Isoniazid (+ Pyridoxine) Pyrazinamide Ethambutol Then R and I for 4 further months

Answer 334

Pyridoxine (vitamin B6) Helps prevent peripheral neuropathy

Answer 335

RI for 3 months Rifampicin Isoniazid (+Pyridoxine) OR Isoniazid for 6 months

Answer 336

R- rifampicin –> red urine/sweat I- isoniazid –> peripheral neuropathy P- pyrazinamide –> gout, hepatitis E- ethambutol –> optic neuritis All can also cause hepatitis

Answer 337

Rifampicin - inhibits bacterial RNA polymerase Isoniazid - Inhibits mycolic acid synthesis Pyrazinamide - Inhibits Fatty Acid Synthetase, disrupting bacterial membrane function Ethambutol - Inhibits cell wall synthesis

Answer 338

R - Liver enzyme inducer I - Liver enzyme inhibitor

Answer 339

Deficit in social interaction, communication and flexible behaviour. Classified under DSM-5/ Uusally present in childhood but may manifest later.

Answer 340

Usually present before 2-3 yo but may manifest later. Impaired social interaction - Lack of eye contact - Delay smiling - Avoids physical contact - Cannot read cues - Difficulty making friends and not wanting to socialise Communication - Delay, absence or regression in language development - Lack of appropriate non verbal communication (eyes, smiling etc) - Difficulty with imaginative or sarcastic behaviour - Repetitive use of phrases or words Behaviour - Interest in patterns, objects, numbers > people - Sterotypical repetitive movements (hand flapping etc) - Intense, deep interests that are persistent and rigid - Anxiety and distress outside normal routine - Extremely restricted food preferences Also associated with higher head circumference to brain volume ratio

Answer 341

Goals: Increase functional independance and QOL: - Learning, development, social skills - Decrease disability/morbidity - Aid families Non pharm: - Educational and behavioural interventions - Family support and counselling Pharm: - SSRIs: Reduce symptoms like stereotyped behaviour, anxiety - Antipsychotics if aggression or self injury - Methylphenidate if associated ADHD

Answer 342

Most common cause of child admission to psych. Patient believes themselves to be fat, despite low or normal weight. DSM-5 criteria - Restriction of energy intake leading to significantly low body weight - Intense fear of gaining weight, despite underweight - Disturbance in the way their body is perceived

Answer 343

Hypogonadism and amenorrhoea - Lack of gonadotrophin (LH/FSH) from pituitary, hypogonadism in ovaries. Cardiac - Arrhythmia, cardiac atrophy, sudden cardiac death. Low BMD Highest mortality of ANY psych condition

Answer 344

Individualised eating disorder focused CBT Maudsley anorexia nervosa treatment for adults (MANTRA) Anorexia focused family therapy in children and young people (CBT 2nd line)

Answer 345

- Low FSH, LH, oestrogen, testosterone - Hypokalaemia - Low T3 - Hypercholesterolaemia - Impaired glucose tolerance G's and C's raised: growth hormone, glucose, salivary glands, cortisol, cholesterol, carotinaemia (orange coloured skin (carrot lol)) Most other things low

Answer 346

Binge eating followed by purging, but inducing vomiting or taking laxatives. Unlike anorexia, body weight tends to stay roughly normal. Lack of control during eating episodes, and associated compensatory behaviours (vomiting, laxatives, exercise etc), occuring at least 1nce weekly for 3 months. Self evaluation unduly influenced by body shape/weight

Answer 347

Normal(ish) body weight - Erosion of teeth - Swollen salivary glands - Reflux - Mouth ulcers - Calluses on knuckles from scraping on teeth (Russell's sign) Alkalosis if repeat vomiting

Answer 348

Normally, testes develop in abdomen, migrate down through inguinal canal and into scrotum. In 5% of boys, they may not descend (cryptochoridsm). May be palpable in inguinal canal. Can lead to testicular torsion, infertility and testicular cancer.

Answer 349

Most will descend in firt 3-6 months. If not, orchidopexy. If bilateral, review urgently.

Answer 350

Testicles that move back into inguinal canal when cold or cremasteric reflex is triggered.

Answer 351

Medical emergency Twisting of spermatic cord causing ischaemia and necrosis. - Severe sudden pain that may refer to lower abdo. - Swollen tender testis retracted upwards (high riding) - Lost cremasteric reflex - Elevation doesnt ease pain (Prehn's sign)

Answer 352

Emergency surgery within 6 hrs. Bilateral detorsion and orchidopexy (fixing to scrotal sac) If unviable, Orchiectomy Manual detorsion if surgery not possible for 6 hrs

Answer 353

As part of an X linked recessive trait, Kallmann's is a cause of delayed puberty secondary to hypogonadotrophic hypogonadism. Thought to be failure of GnRH-secreting neurones to migrate to hypothalamus

Answer 354

TP - Boy with lack of smell and delayed puberty Presentation - Delayed puberty and lack of smell - Hypogonadism, cryptochordism - Low sex hormones - LH, FSH levels low - (normal height) - Cleft palate, visual/hearing defects in some patients

Answer 355

Testosterone supplementation Gonadotrophin supplementation may result in sperm production if fertility desired

Answer 356

8-14 in girls 9-15 in boys Both take about 4 years and girls usually have pubertal growth spurt first. In girls, breast buds -> pubic hair -> menstrual periods (~2 years post start) In boys, enlargement of testicles -> then of penis -> darkening of scrotum -> pubic hair and deepening of voice Staged with tanner staging

Answer 357

Hypogonadism - Lack of sex hormones (oestrogen or test), that normally rise just before puberty, causing a delay. Can be hypogonadotrophic (deficiency of LH and FSH) or hypergonadotrophic (lack of response to LH and FSH by gonads)

Answer 358

Gonads fail to respond to stimulation. Lack of negative feedback means anterior pituitary produces increased LH and FSH. - Damage to gonads (T Torsion, Cancer, Infections) - Congenital absence - Kleinfelter's (XXY) - Turner's (XO)

Answer 359

Start investigations in boys at 14 or girls at 13 if no evidence of puberty, OR no progress in 2 years. Investigate for - Anaemia/Coeliac - Early morning LH/FSH - Thyroid function - IGF-1 (GH deficiency check) - Prolactin - X ray of wrist (Bone density) - MRI brain (pituitary/olfactory bulbs (Kallmann))

Answer 360

development of secondary sexual characteristics before 8 years in females and 9 years in males. <8yo in girls <9yo in boys More common in girls

Answer 361

Girls - Thelarche (Breast development) - Pubic (adrenarch)/underarm hair growth - Menarche - widening of hips and body fat distribution Boys - Facial, pubic, underarm hair - Deep voice - Enlarged testes/penis - Increased muscle mass

Answer 362

Gonadotrophin dependent (central/true) - Premautre activation of HPG axis - FSH and LH RAISED Gonadotrophin independent (pseudo/false) - Excess sex hormones - FSH and LH LOW

Answer 363

Central - Idiotpathic (Most common, esp girls) - Brain lesions (tumours, infections, malformations e.g. hydrocephalus, radiation) - Mutations Peripheral - Less common, moreso have a cause (tumour etc) - Ovarian cysts/tumours - Testicular tumours Bilateral test enlargement - GNRH from intracranial lesion Unilateral - Gonadal tumour Small - Adrenal cause (tumour/hyperplasia) In girls it is normally idiopathic and follows normal course

Answer 364

Autosomal recessive deficiency in cortisol and aldosterone production, and overproduction of androgens from birth. Lack of cortisol = extra ACTH = excess adrenal androgens, causing virilisation of females and young males.

Answer 365

21 hydroxylase converts progesterone to aldosterone and cortisol. Progesterone is also used to make testosterone, but this conversion does not rely on the 21-hydroxylase enzyme. In CAH, there is a deficiency of 21-hydroxylase. Hence, the extra progesterone gets converted to testosterone, causing patient to have low aldosterone, low cortisol and abnormally high testosterone.

Answer 366

Anterior pituitary responds to low levels of Cortisol by producing extra ACTH. A byproduct of this production is Melanocyte Stimulating Hormone production, stimulating production of melanin in cells

Answer 367

Most common: 21-hydroxylase deficiency - Impairs conversion of 17-hydroxyprogesterone to 11-deoxycortisol, causing cortisol deficiency and excess androgen production 11-beta hydroxylase deficiency - Results in HTN due to excess deoxycorticosterone 17-hydroxylase deficiency - mineralocorticoid excess and low androgen and estrogen levels

Answer 368

Depend on deficiency and severity. Virilisation - Excessive androgen exposure makes females present with ambiguous genitalia and male features Salt wasting crisis - Dehydration, hypotension, electrolyte imbalances - 75% of 21-hydroxylase deficiency Precocious puberty Infertility Height and weight acceleration, deep voice, facial hair, absent periods, large penis, small testicles Skin hyperpigmentation (due to excess ACTH causing melanocyte stimulating hormone release)

Answer 369

ACTH stimulation testing - Evaluates adrenal gland's response to ACTH, with abnormal increase in 17-hydroxyprogesterone indicating CAH

Answer 370

Hydrocortisone (glucocorticoid) - Reduce ACTH levels and minimise androgen production Fludrocortisone if mineralocorticoid deficiency Females with virilisation may need surgery

Answer 371

Glucocorticoids e.g. cortisol, main stress hormone. Reduce inflammation, raise glucose, suppress immune system. Levels fluctuate, highest in morning and during stress. Released in response to ACTH from anterior pituitary Mineralocorticoids e.g. Aldosterone. Act on kidneys to control salt and water balance. Released by adrenal in response to renin. Aldosterone acts on kidneys to increase sodium reabsorption and potassium excretion from blood.

Answer 372

Hypothalamic Hamartoma - Benign, associated with gelastic (laughing) seizures and precocious puberty Craniopharyngioma - Benign, derived from Rathke's pouch, cause growth failure, delayed puberty, diabetes insipidus Gliomas - Usually cause visual disturbance and hydrocephalus due to CSF obstruction and associated with neurofibromatosis type 1 Germinomas - Hypopituitarism, precocious puberty, DI

Answer 373

X linked recessive condition. End organ resistance to test causes genotypically male (46XY) children to have female phenotype. Presents with: - Primary amenorrhead - Little/no axillary and pubic hair - Undescended tests -> groin swelling - Breast development (testosterone -> oestradiol) Buccal smear or chromosomal analysis. Manage with orchidectomy

Answer 374

Consider intervention if BMI at 91st centile or above Consider comorbidity assessment if 98th centile or above Overweight if BMI >85th centile and obese >95th centile

Answer 375

- Asian - 4x more likely - Female - Taller children

Answer 376

Lifestyle factors! GH deficiency Hypothyroidism Down's Cushing's Prader-Willi syndrome

Answer 377

Ortho problems - Slipped upper femoral epiphyses - Blount's disease (developmental abnormality of tibia -> bowed legs) Psychological consequences - Bullying, low self esteem Sleep apnoea Benign intracranial HTN Longer term - T2DM, HTN, IHD

Answer 378

Weight loss in first 5 days can be normal Breast fed ~10% Formula fed ~5% Should be back to birth weight by day 10

Answer 379

Tisomy 21 (3 copies), causing characteristic physical features e.g. flattened facial profile, upward slanted eyes, single palm crease. Also presents with intellectual disability, developmental delays, and increased risk of certain conditions.

Answer 380

In order of commonness: Endocardial cushion defect (AV Septal canal defects) Ventricular septal defect Atrial septal defect Tetralogy of fallot Patent ductus arteriosus

Answer 381

Single palmar crease Hypotonia Congenital heart defects Duodenal atresia Hirschprung's disease (aganglionic bowel = constipation)

Answer 382

- Sub/infertility - Learning difficulties - Short stature - Repeated resp infection and glue ear - Hypothyroidism - Alzheimer's - ALL - Atlantoaxial instability

Answer 383

Best: Combined test (11-14 weeks) - USS measures nuchal thickness (back of fetal neck). >6mm is significant. - bHCG. Higher=greater risk - PAPP-A (pregnancy associated plasma protein A) - Lower result=greater risk Triple and Quadruple tests also done if booked later (14-20 weeks) T: bHCG, AFP (lower=risk), and serium oestriol (lower=risk) Q: adds inhibin A (higher=risk) All bloods are maternal

Answer 384

If higher risk (>1 in 150), either second screening, or diagnostic test. NIPT - second screening but non invasive, so may be preferred. Analyses cell free fetal DNA (cffDNA) in maternal blood. 99% sensitivity and specificity Diagnostic tests: Chorionic Villus Sampling - USS guided biopsy of placental tissue <15 weeks Amniocentesis - later in pregnancy, when enough amniotic fluid to take safe sample

Answer 385

- Thyroid (2 yearly) - Echocardiogram - Regular audiometry and eye checks Occupational therapy Speech and language therapy Physiotherapy Dietician Paediatrician GP Health visitors Cardiologist for congenital heart disease ENT specialist for ear problems Audiologist for hearing aids Optician for glasses Social services for social care and benefits Additional support with educational needs

Answer 386

Trisomy 21 - Downs Trisomy 18 - Edwards Trisomy 13 - Pataus

Answer 387

Pataus - Microcephalic, small eyes - Cleft lip/palate - Polydactyly - Scalp lesions Edwards - Micognathia (small lower jaw) - Low-set ears - Overlapping of fingers - Rocker bottom feet (convex foot bottom - present in both)

Answer 388

E: Low HCG, Oestriol and AFP NTD: Raised AFP

Answer 389

When a male has an additional X chromosome, becoming 47XXY instead of the normal 46XY Extra X chromosomes (48XXXY etc) can occur, having more severe features Chromosomal Nondisjunction

Answer 390

Develop as normal until puberty, then develop features - Taller - Wider hips - Gynaecomastia - Weak muscles, small testicles, reduced libido - Shyness - Infertility - Low test but high gonadotrophin Life expectancy close to normal. Slight increase in risk of: - Breast cancer (still less than F) - Osteoporosis - Diabetes - Anxiety/depression

Answer 391

When a female has a single X chromosome, making them 45 XO. (O refers to an empty space). Caused by either presence of only 1 X or deletion of short arm of one. - Short stature, webbed neck, widely spaced nipples - Cubitus valgus (when arm is extended down with palm facing out, angle of forearm is exaggerated) - Underdeveloped ovaries with reduced function (infertile) - Late/incomplete puberty - Primary amennorhoea - Lymphoedema in neonates (feet) - Horseshoe kidney - Bicuspid aortic valve causing ejection systolic murmur

Answer 392

Trinucleotide repeat disorder, affecting FMR1 (Fragile X Mental Retardation 1) gene on X chromosome. (CCG) Affects boys much more severely than girls

Answer 393

X linked recessive inherited disorder or dystrophin genes required for normal muscle function. - Progressive proximal muscle weakness from 5 years. - Calf pseudohypertrophy - Gower's sign: Child uses arms to stand up from squat position

Answer 394

- Raised CK - Genetic testing definitive Prognosis - Most cant walk by 12 - 25-30 yo life expectancy - Associated with Dilated Cardiomyopathy

Answer 395

Autosomal dominant condition (C12 maybe?) "Male Turners" - Webbed neck, widely spaced nipples, short stature, pectus carinatum and excavatum - Ptosis - Tringular face - Lowset ears - Factor XI deficiency and pulmonary valve stenosis

Answer 396

Prader-Willi Syndrome is a loss functional genes on the proximal arm of chromosome 15. Can be due to a deletion of chromosome, or when both are inherited by mother. Called Angelman syndrome if deletion is from mother (or both inherited from father)

Answer 397

Hypotonia during infacny Constant hunger that leads to obesity Dysmorphic features Hypogonadism and infertility Short stature

Answer 398

Growth hormone, aimed at improving muscle development and body comp.

Answer 399

Deletion of MATERNAL chromosome 15, or inheritance of both from father. Like Prader Willi but the other way round.

Answer 400

Random microdeletion on chromosome 7. - Starburst eyes - Wide mouth/big smile - Small chin - Elfin-like facies - Very friendly and sociable - Short stature

Answer 401

Supravalvular aortic stenosis ADHD Hypertension Hypercalcaemia (low calcium diet in management)

Answer 402

Group of disorders affecting collagen formation, cause bones to be brittle. Autosomal Dominant - Hypermobility - Blue sclera - Fractures following minor trauma - Deafness seconday to otosclerosis - Dental imperfections

Answer 403

Calcium, phosphate, PTH, ALP usually normal

Answer 404

Bisphosphonates to increase BMD Vit D supplementation

Answer 405

Inadequately mineralised bones, causing soft and easily deformed bones. (osteomalaica, but in kids) Usually due to vit D deficiency - Dietary deficiency of calcium (e.g. developing countries) - Prolonged breastfeeding - Unsupplemented cows milk formula - Lack of sunlight

Answer 406

Abnormalities - Genu varum (bow legs) - younger kids - Genu valgum (knock knees) - older kids Also: - Aching bones/joints - Rickety rosary (swelling of costochondral junction) - Kyphoscoliosis - Crainotabes (soft skull delayed closure of sutures) - Harrison's sulcus

Answer 407

Serum 25-hydroxyvitamin D - low X ray - osteopenia (radiolucent bones) Calcium and phosphate maybe low ALP and PTH maybe high

Answer 408

Oral vit D - Ergocalciferol + calcium supplementation *Bow legs normally self resolve by 4

Answer 409

Transient inflammation of the synovial membrane of the hip following a viral URTI. Most common cause of hip pain in children aged 3-10. - Limping - Refusal to weight bear - Hip/groin pain - NO/LOW GRADE FEVER (If fever, treat for septic!)

Answer 410

- Exclude septic ASAP. If generally unwell, admit. Otherwise, significant improvement within 48 hours, with full recovery in 1-2 weeks.

Answer 411

Infection within a joint. Most common in under 4s. Emergency, can destroy joint and cause sepsis. Common complication of joint replacement

Answer 412

Single, hot, swollen, red joint Non weight bearing Stiffness and reduced motion Systemic symptoms (fever, lethargy, malaise)

Answer 413

Staph aureus N gonorrhoea (sexually active) Group A strep (pyogenes - under 5) H influenzae E coli

Answer 414

Joint aspiration - Yellow/cloudy, high WCC, culture positive, neutrophils - Send for gram staining, culture, antibiotic sensitivity and crystal microscopy - Take before antibiotics Blood culture. 3-6 weeks,

Answer 415

1. Fever>38.5 (high grade) 2. Non weight bearing 3. Raised ESR 4. Raised WCC

Answer 416

Infection of bone/marrow Haematogenous - Results from bacteraemia, monomicrobial - Most common form in children - Vertebral osteomyelitis is most common in adults Non haematogenous - Contiguous spread from adjacent infection; tissues, bone, direct injury/trauma to bone - Most common form in adults, often polymicrobial

Answer 417

Haem - Sickle cell, IV drug use, immunosuppression, IE Non haem - Diabetic foot, DM, PAD, penetrating injury, cellulitis

Answer 418

Haem - Sickle cell, IV drug use, immunosuppression, IE Non haem - Diabetic foot, DM, PAD, penetrating injury, cellulitis

Answer 419

Staph aureus most common P aeurginosa (gram negative rod) Salmonella (important in sickle cell) N gonorrhoea M Tuberculosis

Answer 420

MRI, X ray can be normal, but possible signs are: - Involucrum/sequestrum (fallen leaf sign) - Periosteal thickening - Lytic lesions - Cortical bone less

Answer 421

6 weeks flucloxacillin Clindamycin if allergic Surgical debridement may be needed (abscess, failure to respond, vertebral osteomyelitis)

Answer 422

Rare hip condition seen usually in obese boys, where head of femur is displaced along growth plate, postero-inferiorly. ~12 years.

Answer 423

Hip pain, can be longstanding, usually following minor trauma (disproportionate to pain) - High, groin, thigh, knee pain - Leg preferred in external rotation, restricted internal rotation due to pain. - Painful limp - Can be bilateral or unilateral

Answer 424

X ray normally diagnostic (antero-posterior and lateral, frog leg views) Managed surgically with internal fixation, with a single cannulated screw in the centre of the epiphysis

Answer 425

Osteoarthritis Avascular necrosis of femoral head Chondrolysis Leg length discrepancy

Answer 426

Inflammation at the tibial tuberosity, where the patella ligament inserts. Common cause of anterior knee pain. Tibial tuberosity is at epiphyseal plate. Stress from running, jumping etc causes apophytis (ligament inflammation). Multiple avulsion fractures, where patella pulls away bits of bone, causing a visible lump under the knee. This starts hard and tender but eventually heals to be non tender.

Answer 427

Supportive - Ice - Reduction in activity - NSAID Physiotherapy after to strengthen joint Complication: Full avulsion fracture - tibial tuberosity is pulled away from rest of tibia

Answer 428

Congenital hip abnormality causes instability and potential for subluxation (partial) or full dislocation. Can lead to weakness, recurrent dislocation, abnormal gait, or early degenerative changes. Usually picked up during newborn examination or when child presents with hip asymmetry.

Answer 429

- First degree family history - Breech from 36 weeks - Breech at birth if 28 weeks - Multiple pregnancy - Female - Firstborn

Answer 430

During NIPE Exam 6-8 weeks. Features suggesting DDH: - Different leg lengths - Restricted hip abduction on one side - Significant bilateral restriction in abduction - Different in knee level when hips flexed - Clunking of hips during special tests If breech, do USS at 6 weeks ALWAYS

Answer 431

Ortolani test - baby on back with knees and hips flexed. Palms on baby's knees with thumbs on inner thigh and four fingers on outer thigh. Gently abduct and apply pressure behind legs to see if legs dislocate anteriorly Barlow test - Baby on back with hips adducted and flexed to 90 and knees bent to 90. Gentle downward pressure applied to knee to see if femoral head will dislocate posteriorly. (clicking is a common finding -> hip will be normal on USS. Clunking more likely to indicate DDH, requires USS)

Answer 432

Most will stabilise by 3-6 weeks If <6 months of age, Pavlik harness is fitted and kept on permanently, holding the femoral head in the correct position and allowing acetabulum (hip socket) to take correct shape. Pavlik harness keeps hips flexed and abducted. Usually removed after 6-8 weeks.

Answer 433

A congenital abnormality where the meniscus (usually C shaped) ends up disc shaped, commonly affects lateral meniscus. 6-10 years Makes the meniscus broader, thicker and more circular causing pain, swelling, clicking, decreased range of motion and locking/giving way of the knee

Answer 434

- MRI is Gold standard imaging Untreated: - Early onset osteoarthritis - Post meniscectomy: pain, instability, join degeneration etc

Answer 435

Inflammatory arthritis lasting >6 weeks in someone younger than 16. If there are systemic symptoms its called Still's disease

Answer 436

ANA may be raised (associated with anterior uveitis) RF negative Raised: CRP, ESR, platelets and ferritin

Answer 437

Oligoarticular - 1 joint, girls under 6, anterior uveitis (refer to ophthalmology) ANA positive Polyarticular - >5 joints, symmetrical, may have fever, anaemia, reduced growth Enthesitis related - Involves inflammation where tendon inserts into muscle. HLAB27, psoriasis, anterior uveitis. Psoriatic - Skin psoriasis and associated hand changes (pitting, onycholysis, dactylitis, enthesitis)

Answer 438

Paediatric rheumatology - NSAID - Steroids - DMARD (methotrexate etc) - Biologics (Anti-TNF etc)

Answer 439

- Lateral (coronal plane) spinal curvature >10 degrees, often has vertebral rotation. - Affects females most and can be congenital, neuromuscular, syndromic (marfans, ehlers danlos) - Affects females more - Uneven shoulders, hips, ribcage asymmetry, back pain - Adams forward bend test, rib hump on side of bend. - Cobb angle>10 = scoliosis. >50 progress into adulthood.

Answer 440

A condition where the head is tilted one way but rotated to the opposite side. Can be congenital or acquired Presents with head tilt to one side and rotation to other. Palpable mass or tightness in sternocleidomastoid (fibrosis) USS to assess SCM Physio, botulinum toxin, surgery etc for management Good prognosis if physio, resolved by 1 yr

Answer 441

Mild jaundice and scleral ictera from 2-7 days, resolves in 10. Fetal RBC break down quicker, but bilirubin normally excreted via placenta. At birth, no placenta, so rise in bilirubin while liver develops and starts removing bilirubin

Answer 442

ALWAYS pathological Increased production - Haemolytic disease of the newborn - ABO or Rhesus haemolytic disease - Haemorrhage - G6PD deficiency - Sepsis! Decreased clearance - Prematurity - Breast milk jaundice - Neonatal cholestasis - Extrahepatic biliary atresia - Gilbert syndrome

Answer 443

Kernicterus - Bilirubin crosses blood brain barrier causing CNS damage. Presents with less responsive, floppy, drowsy baby with poor feeding. Deafness Developmental delay Acute bilirubin encephalopathy

Answer 444

Haemolytic disease of the newborn (Rhesus disease)

Answer 445

Treatment threshold chart - GA of baby (hours) against total bilirubin Phototherapy first line, extremely high may require exchange transfusion (remove blood from neonate, replace with donor)

Answer 446

Blue light (little/no UV) is used to break down unconjugated bilirubin into isomers, meaning they can be excreted in bile and urine without conjugation. Measure rebound bilirubin 12-18 hours later to ensure no further treatment needed

Answer 447

When contractions occur, the placenta cant carry out gas exchange, leading to hypoxia. Extended hypoxia leads to anaerobic respiration and a drop in heart rate (bradycardia). Further hypoxia reduces consciousness and drop in respiratory effort, worsening hypoxia. Extended hypoxia leads to hypoxic-ischaemic encephalopathy, can cause cerebral palsy.

Answer 448

(First 30 secs) - Dry and warm baby ASAP. - Assess babys tone (floppy?), breathing (chest moving?), HR (Ideally >100bpm) If not gasping/breathing or HR<100 - open airway and give 5 inflation breaths (5 breaths 2/3 secs each, aiming for chest rise) Reassess HR and chest movements - If HR60-100, continue with ventilation breaths 40/60 a min. - If HR<100, begin compressions, at 3 compressions to 1 breath. (90 compressions and 30 breaths per min) - Reassess HR every 30 seconds

Answer 449

Hypoxia Hypovolaemia Hypoglycaemia Hypothermia

Answer 450

APGAR - 1, 5 and 10 mins after birth A - appearance (colour) - 0: Blue/pale - 1: pink, but extremities blue - 2: Pink P - Pulse - 0 No HR, 1 for <100, 2 for >100 G - Grimace (reflex irritability - suctioning, gentle slap on soles of feet etc) - 0: No response, 1: grimace/weak cry, 2: vigorous cry, pull away etc A - Activity - 0: Limp, 1: some flexion of arms/legs, 2: Active motion, well flexed limbs R - Respiration - 0: No breathing, 1: weak, irregular, gasping breaths, 2: Strong, regular breathing, crying >7 = good 4-6 = moderate low <3 = Bad

Answer 451

Delayed cord clamping - significant fetal blood in placenta, allows it time to enter baby circulation. Improves Hb, iron stores, BP, reduces intraventricular haemorrhage, necrotising enterocolitis. Does cause more neonatal jaundice. If they require resus, dont wait.

Answer 452

Premature neonates - usually <32 weeks, before lungs start producing surfactant. Causes high surface tension in alveoli, causing atelectasis (lung collapse) as alveoli and lungs cant expand. Causes inadequate gas exchange, causing hypoxia, hypercapnia, respiratory distress.

Answer 453

X ray shows ground glass appearance (bilateral infiltrates) Management - Antenatal steroids (Dexamethasone) to mother with suspected/confirmed preterm labour, increases surfactant production. - Intubation/ventilation of child - Endotracheal surfactatnt - CPAP - O2 sats maintained 91-95

Answer 454

Short - Pneumothorax - Infection - Intraventricular haemorrhage - Apnoea - Pulmonary haemorrhage - Necrotising enterocolitis Long - Chronic lung disease of prematurity - Retinopathy of prematurity - Neurological, hearing, visual impairment

Answer 455

24-34 weeks gestation, by type 2 alveolar cells

Answer 456

Fluid collection on the scalp, outside the periosteum, and hence, crosses suture lines. Caused by a traumatic, prolonged, or instrumental delivery. Usually no discoloration of the skin, and resolves on its own in days Caput SuccaDAYneum (Crosses Sutures) - resolves within a few days

Answer 457

AKA traumatic subperiosteal haematoma. (between skull and periosteum) Caused by damage to blood vessels in traumatic, prolonged, instrumental delivery. Presents as discolouration and lump, which does not cross suture lines. Resolves on its own over months. Risk of anaemia and jaundice. cephalohaematoMONTH (doesn't cross sutures) - resolves within a few months

Answer 458

Facial paralysis - Caused by damage to facial nerve, one sided symptoms. Function returns on its own in a few months

Answer 459

Damage to C5/C6 nerves in brachial plexus during birth. Associated with shoulder dystocia, traumatic/instrumental birth, or high birth weight. Leads to weakness of shoulder abduction and external rotation, arm flexion and finger extension. Causes waiters tip deformity: - Internally rotated shoulder - Extended elbow - Flexed wrist pronated (facing backwards) - Lack of movement. (as if someone is discretely trying to take something being passed from behind) Function returns spontaneously in months

Answer 460

- Events that could lead to hypoxia during the perinatal or intrapartum period - acidosis (pH < 7) on the umbilical artery blood gas, - poor Apgar scores - Multi organ failure Caused by: - Maternal shock - Intrapartum haemorrhage - Prolapsed cord - Nuchal cord (cord wrapped around baby neck)

Answer 461

Mild: poor feeding, generally irritable, hyper alert. Resolves in 24 hrs Moderate: Lethargy, hypotonic, seizures. Takes weeks to resolve. 40% Cerebral palsy Severe: Reduced consciousness, apnoea, flaccid, absent reflexes. 50% mortality, 90% palsy

Answer 462

When baby is close to term. Target 33-34C, measured with rectal probe. 72 hrs, then rewarmed over 6 hrs. Reduces inflammation and neurone loss after initial injury. Reduces risks of complication

Answer 463

Respiratory distress in newborn caused by meconium in trachea. More common post term (44%>42 weeks). Higher rates in - Maternal HTN - Pre eclampsia - Chorioamnionitis - Smoking - Substance abuse

Answer 464

Vitamin K! IM in thigh shortly after birth. OR orally, immediately, 7 days and 6 weeks

Answer 465

Blood spot screening - Day 5-9. Heel prick gets blood drops. - Sickle cell - CF - Congenital hypothyroid - Phenylketonuria - MCADD - MSUD - IVA -GA1 - Homocystin

Answer 466

Chronic lung disease affecting premature infants, especially those on oxygen therapy. Results from lung injury caused by oxygen, pressure from ventilation and inflammation.

Answer 467

- Fewer, larger, simplified alveoli. - Ventilator induced airway pressure damage and overdistension of alveoli - Oxygen toxicity (rich oxygen species damage lung cells including alveolar epithelium and endothelial cells of blood vessels. - Inflammation

Answer 468

CXR - lung overinflation, atelectasis History of O2/ventilation need >36 weeks in preterm infant. - Pulmonary HTN - Recurrent infection - Developmental delay - Growth retardation - SIDS

Answer 469

TORCH T - Toxoplasmosis Other (Syphilis, VZV, HIV) R - Rubella C - Cytomegalovirus H - Herpes simplex

Answer 470

Toxoplasma gondii (often from contaminated food or cat faeces) Obligate intracellular protozoan

Answer 471

Rubella CMV Herpes simplex All viral

Answer 472

Neurological - Cerebral calcification - Hydrocephalus - Chorioretinitis Ophthalmic - Retinopathy - Cataracts

Answer 473

Pyrimethamine plus sulphadiazine, 6 weeks Ring enhancing lesions

Answer 474

T: Chorioretinitis, hydrocephalus, intracranial calcifications R: Cataracts, Congenital Heart defects, sensorineural hearing loss, developmental delays CMV: Sensorineural hearing loss, microcephaly, jaundice, hepatosplenomegaly HSV: Vesicular skin lesions, encephalitis

Answer 475

- Serology on maternal and infant blood (IgM, IgG) - PCR testing for viral DNA - USS (Fetal abnormalities - hydrocephalus, calcifications) - Amniocentesis

Answer 476

Bowel disorder affecting premature neonates. Part of the bowel becomes necrotic and can lead to perforation, peritonitis, shock and sepsis

Answer 477

X ray - Dilated bowel loops - Bowel wall oedema - Pneumatosis intestinalis (intramural gas) - Pneumoperitoneum (free gas in peritoneal cavity - implies perforation) - Air both inside and outside bowel wall

Answer 478

NBM IV fluids TPN ABx Surgical emergency - short bowel syndrome is a complication.

Answer 479

Congenital defect in abdominal wall causes organs to protrude directly through the hole. This normally presents to the right of the belly button. Requires surgical correction, should be covered with cling film in mean time

Answer 480

Abdominal organs protrude through hole to right of belly button. No protective covering, meaning they can be irritated by amniotic fluid. Isolated defect, no association More common in young (teenage) mothers. Good prognosis after surgery

Answer 481

AKA Exomphalos Abdominal organs protrude through abdominal wall (usually at the belly button), but are covered by an amniotic sac, made of amniotic membrane and peritoneum. Unlike gastroschisis, has some associations, and associated with advanced maternal age.

Answer 482

- Bekwith-Wiedemann syndrome - Downs (Trisomy 21, but also, 13 and 18) - Cardiac and kidney malformations

Answer 483

C section indicated to reduce risk of sac rupture. Staged repair - to allow lung development - Sac allowed to granulate and epithelialise - Forms a shell, when child big enough, contents can be put back into abdomen.

Answer 484

Bowel (usually small bowel) - Duodenal, Jejunal, Colonic Oesophageal - Oesophageal atresia with tracheosophageal fistula (TEF) most common - Can be isolated atresia - Results in a blind pouch or disconnected oesophagus

Answer 485

Bowel - Bilious vomiting - Abdominal distension - Failure to pass meconium Oesophageal - Drooling/choking post birth - Difficulty swallowing - Coughing, gagging, cyanosis - Abdo distension

Answer 486

Prenatal USS - Bowel: Polyhydramnios and bowel loops. - Oesophageal: Polyhydramnios X ray: - Bowel: Dilated bowel loops. Double bubble sign in duodenal. - Oesophageal: Confirm diagnosis - blind ending oesophagus. Air in stomach if TEF OA will block NG tube being passed to stomach

Answer 487

Oesophageal - impaired swallowing of amniotic fluid. Also seen in duodenal atresia, less frequently in other bowel atresias.

Answer 488

Downs (duodenal) CF (jejunal/ileal atresia)

Answer 489

VACTERL abnormalities - Vertebral - Anorectal - Cardiac - Transoesophageal - Renal - Limb - Downs, Trisomy 18

Answer 490

Nasogastric decompression and fluid resuscitation (+prevention of aspiration in oesophageal) Oesophageal: GORD, Oesophageal strictures, TEF Bowel: Short bowel syndrome (if resected), malabsorption, need for nutritional support

Answer 491

Duodenal atresia - Few hours post birth, AXR shows double bubble sign Malrotation with volvulus - Incomplete rotation during embryogenesis, 3-7 days post birth - Emergency, volvulus may cut off blodd supply, causing peritoneal signs - Treat with Ladd's procedure Necrotising enterocolitis - 2nd week of life - Dilated bowel loops, pneumatosis, portal venous air Meconium ileus - 24-48 hrs, associated with abdo distension, CF - Air-fluid levels on AXR

Answer 492

Infection in neonatal period. Non specific presentation; low threshold for treatment and high suspicion. Most common in late preterm infants - Early (<72 hrs) Group B strep (Lives harmlessly in maternal vagina) Late onset (7-28 days - post delivery environment, contact from parents) - E coli (2nd most) - Staph epidermidis - Pseudomonas - Klebsiella - Enterobacter - Staph aureus

Answer 493

- Vaginal GBS - GBS sepsis in previous birth - Maternal sepsis, chorioamnionitis, fever>38C - PROM - Early rupture of membranes - Prematurity - Current maternal UTI

Answer 494

Non specific - Resp distress (grunting, nasal flaring, use of accessory muscles, tachypnoea) - Tachycardia - Jaundice - Seizures (if meningitis) - Vomiting - Temperature (Can be high or low)

Answer 495

- Blood culture definitive. Ideally 2 cultures, to distinguish contamination - FBC, CRP - Blood gases (metabolic acidosis concerning) - Urine MCS - Lumbar puncture

Answer 496

- IV Benzylpenicillin with gentamicin, first line. - Remeasure CRP 18-24 hours after presentation, if <10 and negative culture, cease Abx Maintain - O2 sats - Fluid/electrolyte status - Prevention of hypoglycaemia and metabolic acidosis

Answer 497

Tactile stimulation IV Caffeine

Answer 498

Normal term often have hypoglycaemia in first 24 hours, which is usually fine because they can use ketones and lactate as fuel. ~<2.6mmol/L Transient hypoglycaemia common in the few hours post birth!

Answer 499

May be asymptomatic - no treatment just breast feeding and monitoring. Autonomic - Jitteriness - Irritability - Tachypnoea - Pallor Neuroglycopenic - Poor feeding/sucking - Weak cry - Hypotonia - Seizures - Drowsy - Apnoea and hypothermia If symptomatic, or very low, - Admit to neonatal unit and IV infusion of 10% dextrose!

Answer 500

1 in 20. 87% of these are gestational diabetes. 2nd most complicatin of pregnancy after HTN.

Answer 501

Oral Glucose Tolerance Test - ASAP After booking, again at 24-28 weeks. - Fasting >5.6, 2-hour glucose >7.8

Answer 502

See in joint diabetes and antenatal clinic within week If fasting glucose <7 - diet and exercise - If doesnt work in 1-2 weeks, start metformin - If that doesnt work add SHORT ACTING insulin If fasting glucose >7m start insulin Glibenclamide if cannot tolerate metformin, or decline insulin

Answer 503

- Weight loss to BMI 27 - Stop all meds except metformin and start insulin - Folic acid 5mg/day, pre conception to 12 weeks - Detailed anomaly scan, including 4 heart chambers - Retinopathy worsens during pregnancy

Answer 504

Fasting <5.3 1 hr post meal <7.8 2 hr post meal <6.4

Answer 505

- Thyroxine safe in pregnancy, crosses placenta so need to increase dose by up to 50% - Serum TSH measured each trimester and 6-8 weeks post partum - Safe to breastfeed on thyroxine - Complications include, miscarriage, anaemia, SGA, pre-eclampsia

Answer 506

There is an increase in thyroxine-binding globulin, increasing total thyroxine but not free thyroxine

Answer 507

Increases risk of - Fetal loss - Maternal heart failure - Premature labour

Answer 508

Transient Gestational Hyperthyroidism TSH receptor activated by HCG - increases in first trimester, decreases in 2 and 3

Answer 509

Propylthiouracil! (Risk of severe hepatic injury though) Carbimazole associated with increased risk of congenital abnormality so not used Keep maternal free thyroxine in upper 3rd of normal, so fetus gets enough thyroid hormone Thyrotrophin receptor stimulating antibodies checked 30-36 weeks NO RADIOIODINE

Answer 510

1in1000. Most common congenital abnormality affecting orofacial. - Polygenic inheritance - Maternal antiepileptic use increases risk - Lip results from failure of fronto-nasal and maxillary processes to fuse - Cleft palate from failure of palatine processes and nasal septum to fuse

Answer 511

Feeding: Orthodontic devices Speech: speech therapy Increased risk of otitis media

Answer 512

Lip repaired first first week-3 months Palate repaired 6-12 months

Answer 513

Strep agalctiae. Transferred from mother to baby during labour. Harmless in maternal vagina but can cause serious infection; Sepsis, pneumonia, respiratory distress, meningitis Late onset (7-89 days): Meningitis, bacteraemia, focal infections (bone joint tissue)

Answer 514

Recto-vaginal swab for GBS at 35-37 weeks Intrapartum antibiotics for at risk or confirmed - Neurodevelopmental impairment (esp meningitis) - High mortality (10-20%)

Answer 515

Listeria monocytogenes (Gram positive rod) - Maternal consumption of contaminated food (Unpasteurised dairy, deli meat, smoked seafood) - Immunocompromised - Preterm birth

Answer 516

Blood culture CSF culture Placental/cord culture CSF analysis shows elevated protein, low glucose, increased WBC (pleocytosis)

Answer 517

Sepsis, meningitis, pneumonia

Answer 518

IV Amoxicillin or ampicillin + Gentamicin

Answer 519

Encephalitis caused by HSV-1 Usually attacks the temporal lobes, but also inferior frontal

Answer 520

- Fever, headache, psychiatric symptoms, seizures, vomiting - Focal temporal lobe signs: Anterograde/Retrograde amnesia, Auditory hallucinations, Aphasia (Wernicke's - posterior part of superior temporal gyrus), emotional changes, recognition changes

Answer 521

- CSF: lymphocytosis, elevated protein - PCR for HSV - CT: Medial temporal and inferior frontal changes (e.g. petechial haemorrhages). MRI preferred. - EEG lateralised period discharges at 2Hz Managed with aciclovir

Answer 522

Newborn - Otoacoustic emission test Infants - Auditory brainstem response test 6-9 months - Distraction test <2.5yo - Recognition of familiar objects >2.5yo - Performance or speech discrimination testing >3yo - Pure tone audiometry

Answer 523

when HR <60bpm (bradycardic). Start with 5 rescue breaths, then 15 compressions: 2 breaths. 100-120/min, lower half of sternum.

Answer 524

Meconium hasnt passed. CF Hirschprungs Big differentials to rule out

Answer 525

Really Sick Babies Get Antibiotics 1. Recessions (moderate or severe chest wall recessions) 2. Skin turgor reduced 3. Blue or mottled appearance 4. Grunting 5. Asleep (does not wake if aroused)

Answer 526

Retinal haemorrhage, Subdural haematoma, Encephalopathy

Answer 527

Self limiting condition caused by coxsackie A16 and enterovirus 71 - Causes mild coryzal symptoms - Oral ulcers - Red vescilces on palms and feet

Answer 528

Diagnosed: Fine toothed combing of wet or dry hair Malathion, wet combing, dimeticone, isopropyl myristate and cyclomethicone, Patient can choose No school exclusion

Answer 529

Relatively common, benign presentation where baby (<3 months) has colic. Self resolves.

Answer 530

Immune/idiopathic reduction in platelet count. Antibodies created against glycoprotein IIb/IIIa or Ib-V-IX complex. Typically more acute in children, and normally follows infection or vaccination. TYPE 2 HYPERSENSITIVITY

Answer 531

Pupuric rash - non blanching spots of bleeding under the skin Bruising Bleeding less common - usually presents as nose or gum bleeding

Answer 532

full blood count - should demonstrate an isolated thrombocytopenia blood film bone marrow examinations is only required if there are atypical features e.g. lymph node enlargement/splenomegaly, high/low white cells failure to resolve/respond to treatment

Answer 533

Usually no treatment required - ITP resolves in 80% of children within 6 months, with or without treatment Avoid trauma (e.g. team sports) If platelet count very low - Steroids, IV immunoglobulins, platelet transfusion EMERGENCY only

Answer 534

More chronic, childrens tends to be acute and self resolves More likely to bleed (catastrophic uncommon) Managed with oral prednisolone IV immunoglobulins raises platelet count faster

Answer 535

ITP associated with autoimmune haemolytic anaemia

Answer 536

Autosomal recessive - Aplastic anaemia, increased risk of AML - Neurological changes - Short stature and thumb/radius abnormalities - Cafe au lait spots (look like spilt tea or coffee)

Answer 537

Acquired (immune mediated - Coombs positive) - Autoimmune: Warm/cold antibody type - Alloimmune: Transfusion reaction, haemolytic disease of the newborn - Drugs: Methyldopa, penicillin Acquired - Microangiopathic haemolytic anaemia: TTP/HUS, DIC, malignancy, pre-eclampsia - Prosthetic heart valves - Malaria - Zieve syndrome: Coombs-negative haemolysis

Answer 538

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

Answer 539

- exomphalos, - congenital diaphragmatic hernia - intrinsic duodenal atresia

Answer 540

The 5 Ts of Cyanotic Heart Disease, Truncus arteriosus (1 trunk), Transposition of the great arteries (2 great vessels), Tricuspid atresia (tri), Tetralogy of Fallot (tetra), Total anomalous pulmonary venous connection (5 words)

Paediatrics Flashcards

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