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PaedsGastro (&Development) Darren McGrugan, Liv Frith. 1 PAEDS GASTRO: PX & CAUSES CONDITIONS TO BE AWARE OF Whatwewill DEVELOPMENTAL MILESTONES covertoday… DEVELOPMENTAL DELAY SUMMARY & QUESTIONS There is a lot to get through if we are taking the whole of paeds gastroenterology and development (including milestones etc) – therefore, we are going to give you a quick whistle stop tour of HIGH YIELD PT and OSCE related facts – that is what you are here for. Not much point in spouting intricacies which you won’t remember unless you revise yourself. Abdo pain is a massively common Px in kids, and there can be very common and reasonable explanations to these, or something more sinister. 2 Paediatric Gastroenterlogy: Px&Causes You could separate out into acute vs chronic causes. I think working in a presentation- wise manner is more logical, truer to life and helpful to make your notes this way, both for OSCEs and for PT. Will go through the most examinable content/ most relevant. Isn’t necessarily all of the things you could come across. Abdo pain is an incred common Px and is too broad, therefore, will be more specific (otherwise now to next week explaining) 3 - HIRSCHSPRUNG’S - INTUSSUSCEPTION - COW’S MILK ALLERGY CONDITIONS TO - COELIAC BE AWARE OF… - PYLORIC STENOSIS - GORD This isn’t an exhaustive list. This is more to cover newer conditions/ more relevant to paeds as opposed to general gastro. Still need to consider Ddx like obstruction, appendicitis, testicular torsion, IBD and all of those things. It just won’t be helpful to you for us to go over them when they will either be covered in another PPALS session or you revise them yourself. 4 PYLORIC STENOSIS -Progressive projectile vomiting -Olive sign -Hypochloraemic, hypokalaemic metabolic alkalosis -USS -Pyloromyotomy 20XX presentation title 5 Pyloric stenosis is a condition that causes hypertrophy of the pyloric sphincter results in narrowing of the pyloric canal. Presentation - Progressive non-bilious projectile vomiting occurring after feeds. (when I say projectile I mean projectile hits the walls / ceiling in some cases) May have a history of formula changing without resolution of symptoms. May have been misdiagnosed with GORD. Other symptoms - poor weight gain (ultimately failure to thrive), constipation, or symptoms of volume depletion (e.g., decreased wet nappies) 4x more common in males. Other risk factors include: prematurity, birth order (first born), maternal FH and erythromycin in the first few weeks. Most commonly occurs in 3-6 week old infants On examination the key finding is the palpable olive-shaped upper abdominal mass – which is the hypertrophied pyloric muscle felt in the RUQ or the 5epigastric region. Other signs are related to volume depletion e.g dry mucous membranes, flat or depressed fontanelles, tachycardia, CRT> 2 Electrolyte disturbances: hypochloraemic, hypokalaemic metabolic alkalosis – due to persistent vomiting Gold standard test is USS – (Criteria is pyloric muscle thickness >3 mm and pyloric canal length >15 mm) DDx: GORD, over-feeding, Cows’ milk allergy Management: Pyloromyotomy is the definitive treatment. For immediate management prior to surgery replacement of fluid and electrolytes. Good prognosis – surgical intervention success rates nearly 100% and post-op complication are negligible 5 HIRSCHSPRUNG’S DISEASE PX - Meconium delay - Chronic constipation from birth - Abdo pain + distention - Vomiting - FTT/ Poor weight gain Main thing to note is the Px. Things like meconium not passing within 24 hours of birth but after aka a delay in passing are seen with this disease. A bit of a spectrum disease, and this is determined by the pathophysiology which I will explain. During development in utero, PNS ganglion cells start high up in the GIT and gradually migrate down to the rectum, helping to form the myenteric plexus. These cells are needed to promote motility and stimulating peristalsis. In Hirschprung’s, the cells do not migrate the whole way to the end. This could leave a small bit of colon aganglionic, or it could leave quite a large proportion this way, therefore, spectrum of severity in Sx. So you get this decreased motility, and the affected section of bowel cannot relax and becomes constricted. You then get proximal distention as poo cannot pass. Can lead to HAEC (systemically unwell, bloody diarrhoea) which is life-threatening emergency (within 2-4 weeks birth). Important to note this is usually diagnosed within first few weeks of life, therefore, if older (months to years) not very likely. FHX big deal. Some genetic associations – read about them if you want, such as links w. Down’s and Waardenburg Syndrome, but not high yield. 6Management - AXR (diagnoses obstruction and demos features HAEC) - Rectal Biopsy – shows if ganglion cells or not. - If HAEC -> Fluid Resus, Abx, NG, Obstruction management. - Definitive -> Surg resection aganglionic section. 6 GORD - Typically presents <8weeks - Vomiting/ regurgitation following feeds - unusual neck posturese.g. excessive crying, - Clinical diagnosis - Management: thickeened formula, trail alginate (e.g. Gaviscon), PPI 20XX presentation title 7 Gastro-oesophageal reflux disease (GORD) is a common condition you would have rd covered in 3 year, however the presentation and management differs slightly in children. In GORD the tone of the lower oesophageal sphincter is too low resulting in uncontrolled reflux of the stomach contents. This is more likely in infants as they have: - short, narrow oesophagus’ - delayed gastric emptying - Liquid diet and high calorie requirement which distends the stomach and increases the pressure gradient between stomach and oesophagus Important differential as it is the commonest cause of vomiting infancy. Presentation - Typically develops before 8 weeks - Vomiting/ regurgitation following feeds – some reguriattion can be normal - Distressed behviour e.g. excessive crying, unusual neck postures, back arching 7- Unexplained feeding difficulties e.g refusing feeds, gagging, choking - Faltering growth – failure to thrive It is a clinical diagnosis. O/E check: - Hydration status - Signs of malnutrition - Assess growth charts to look for faltering growth Risk factors – prematurity, neurological disorders e.g. cerebral palsy, congenital diaphragmatic hernias, congenital oesophageal atresia Management - Advise regarding position during feeds – 30 degrees head-up - Infants to sleep on their backs as per standard guidance to reduce the risk of cot death - Ensure infant is not being overfed (as per their weight) and consider a trial of smaller and more frequent feeds - A trial of thickened formula (for example, containing rice starch, cornstarch, locust bean gum or carob bean gum) - A trail of alginate therapy e.g. Gaviscon – don’t use at the same time as thickening agents - PPI recommended if infants have unexplained feeding difficulties, distressed behaviour and faltering growth Complications - Distress - Failure to thrive - Aspiration/ aspirational pneuomina - Frequent Otitis media - In older children dental erosion may occur - 90% of infants will spontaneously resolve within the first year DDx: Cow’s milk allergy, pyloric stenosis 7 INTUSSUSCEPTION PX - Severe colicky abdo pain - Redcurrant jelly stool - RUQ mass (sausage) - Vomiting - Intestinal obstruction Important to know when things present in kids, as you wouldn’t consider certain differentials once e.g. past a certain age. This is when the bowel “telescopes” in on itself. This causes the mass and stops shit passing through bowel. - Usually occurs in kids from 6 months to 2 years old. Occurs more in boys > girls. Associated w/ preceding or concurrent viral illness (e.g. uRTI). Associated w/ Henoch- Schonlein Purpura, CF, Intestinal polyps, Meckel. Management: - USS initial investigation always. Therapeutic enema used to try and reduce it non- surgically. O/wise surg reduction. Sometimes need resection if gangrenous etc. 8 NeonatalJaundice - Serum bilirubin >85.5micromol/L (5mg/dl) < 24 hrs 24hrs – 14days > 14 days - Physiological jaundice - Prolonged jaundice - Jaundice in the first 24hrs of life is ALWAYS pathological - Causes: - Causes: - Causes: § Biliary atresia § Rhesus haemolytic § Increased RBC § Hypothyroidism disease breakdown § Prematurity § ABO incompatibility § Immature liver § G6PD deficiency § Hereditary - No treatment or further -Management: phototherapy, Spherocytosis investigation required exchange transfusion 20XX presentation title 9 Neonatal jaundice is defined when serum bilirubin is >85.5micromol/L (5mg/dl). This causes yellowing discoloration of the skin and sclera Is very common occurs in between 50% -70% of term neonates – but most cases are physiological Physiological jaundice may result from: - Increased RBC breakdown – in utero the fetus has a high conc of HB (to maximise oxygen exchange) this is broken down releasing bilirubin - Immature liver – not able to process high conc bilirubon Starts at day 2-3 and peaks by day 5 and usually resolves by day 10. Neonates with physiological jaundice are usually well and don’t need any intervention. More common in breastfed babies – so in a neonate jaundice history always ask whether they are bottle or breast fed Physiological jaundice can progress to pathological jaundice if baby is premature or there is an increase in RBC breakdown e.g. extensive bruising or cephalohaematoma following instrumental delivery 9Pathological jaundice - Jaundice in the first 24hrs of life is ALWAYS considered pathological – causes include rhesus haemolytic disease, ABO incompatibility, G6PD deficiency, hereditary spherocytosis - Prolonged jaundice so jaundice that lasts longer than 14 days (or 21 days if premature) need a jaundice screen: Jaundice screen: •conjugated and unconjugated bilirubin: the most important test as a raised conjugated bilirubin could indicate biliary atresia which requires urgent surgical intervention •direct antiglobulin test (Coombs' test) •TFTs •FBC and blood film •urine for MC&S and reducing sugars •U&Es and LFTs Causes of prolonged jaundice - Biliary atresia - Hypothyroidism - Galactosaemia - UTI - Congenital infections e.g. CMV, tocoplasmosis - Prematurity – due to immature liver function – this increases the risk of kernicterus Management - Phototherapy – interpret bilirubin level using a graph that are gestation specific - Exchange Transfusion Complications - Main complication is kernicterus - billirubin-induced brain dysfunction, can result from neonatal jaundice. Bilirubin is neurotoxic and at high levels can accumulate in the CNS gray matter causing irreversible neurological damage. Depending on level of exposure, effects can range from clinically undetectable damage to severe brain damage. 9 BiliaryAtresia - Cause of prolonged neonatal jaundice - Progressive fibro-inflammatory obstruction of the biliary tree à bile flow obstruction - Px: jaundice, pale, stool, dark urine, disturbanceomegaly, appetite and growth - Total bilirubin – normal - Conjugated bilirubin – raised - Requires surgery 20XX presentation title 10 Quick run through biliary atresia – more of a progress test question. Cause of prolonged neonatal jaundice It is the progressive obliteration of a segment or entire biliary tree which leads to the obstruction in the flow of bile. Pathogenesis is unclear but risk factors include infection, Px: jaundice (which extends beyond the 2 weeks, pale stools, dark urine, appetite and growth disturbance, hepatomegaly with splenomegaly between 2-8 weeks of life Investigations: - Do both total bilirubin and conjugated bilirubin – total may be normal whereas conjugated will be abnormally high LFTs including serum bile acids and aminotransferases are usually raised - USS may show distension and tract abnormalities Management - Surgical intervention is the only definitive treatment 10 COELIAC DISEASE PX - aSx, therefore, low investigation threshold. - FTT - Diarrhoea - Fatigue - Weight loss - Mouth ulcers - Anaemia - Dermatitis herpetiformis Probably all know about and remember. For those who don’t long and short is gluten causes autoimmune reaction whereby autoantibodies target epithelial cells of SI causing inflammation. Note in PX: Anaemia can be secondary to an iron, B12 (big one) or folate defiency (due to poor uptake) Dermatitis herpetiformis (itchy, blistering skin rash that appears on abdo). Can get things like periph neuropathy on Px… rarely happens so don’t worry about learning these, just be aware this is caused by B12 deficiency and is associated. Ix - Test all T1DM babies for coeliac as even if no Sx, big link in conditions - Look for anti-TTG or anti-EMA (these are IgA, so CHECK TOTAL IGA LEVELS -> if low this means your coeliac test will be negative even when have condition) - Must keep on gluten for investigation for obvious reasons. - Can do endoscopy with intestinal biopsy (which shows crypt hypertrophy and villous atrophy). - Tx: lifelong w/o gluten is curative. 11 COW’S MILK PROTEIN ALLERGY PX - GI: Abdo pain, bloating/ wind + D&V - Allergic Sx: - Urticaria - Angioedema - Cough/ wheeze/ sneeze - Watery eyes - Eczema Occurs in the < 3 y/o normally. - Involves hypersensitivity to cow’s milk protein. - Can be IgE mediated i.e. rapid 2 hour response or non-IgE so over several days - Commoner in formula babies and those w atopic FHx. - Usually presents when baby weaning from breast -> formula or milk-containing food (i.e. <1 y/o) - Can very rarely get anaphylaxis - Intolerance vs Allergy so baby will get GI Sx but not allergy Sx… Intolerant babies will have Sx but grow and develop. CMPA babies are not tolerant at all. - Intolerance babies outgrow by 2-3 years … use breast milk or hydrolysed formulas and wean to non-CM foods - Start on milk ladder after year - Remember that mum consuming cow’s milk products can pass this on BF. - Management - Skin prick can be done but isn’t. - Avoidance is used or trials of hydrolysed milk/ mum avoiding dairy when BF. - Every 6 months or so try the milk ladder until Sx and over time this should allow normal diet. 12 Vomiting PX - Characteristics of the vomitus – quantity, colour, blood, bilious - Infective versus non-infective - Assoicated symptoms e.g. diarrhoea, fever, abdo pain VOLUME DEPLETION AND ELECTROLYTE DISTURBANCES!!! Signs of volume depletion/ dehydration - Generalised irritability - Tachycardia - Increased CRT - Dry mucous membranes - Sunken eyes - Sunken fontanelle - Decreased skin turgor - Decreased UO 20XX presentation title 13 Very non-specific presentation with many differentials Can you name a few DDx? - DDx: gastroenteritis (viral, bacterial), pyloric stenosis, intussusception, GORD, diabetic ketoacidosis, constipation, UTI, - Assessment – you wanna work out is there an infectious cause or is this a presentation of a serious medical condition. In the history you wanna elicit: - Characteristics of the vomitus – quantity, colour, blood, bilious - Obvious trigger - Assoicated symptoms e.g. diarrhoea, fever, abdo pain Vomiting (+/- diarrhoea) – one of the main worries is volume depletion and electrolyte disturbance more so than in adults Signs and symptoms – vary with age but include: 13- Generalised irritability - Tachycardia, - Increased CRT - Dry mucous membranes - Sunken eyes - Decreased skin turgor - Decreased UO (Hypotension is a late sign – why its not in PEWS) One of the biggest causes is gastroenteritis Those most at risk: - <1yrs - Infants with low birth weight - Passing >6 diarrhoeal stools or vomiting more than 3 times in 24hrs - Those with existing health issues Mostly diagnosed clinically but can support findings with bloods like U&Es, FBC ect Management - Continue breastfeeding or giving fluids - Oral rehydration solutions 13 Constipation PX o Consider THEIR normal o < 3 stools/ wk. Abdo pain. Straining w hard stool. PR bleeding. Palpable stool. o Overflow soiling. CAUSES o Lifestyle -> PO intake/ Low fibre. Sedentary. Psychosocial. o Secondary -> Hirschsprung’s/ CF/ Intestinal Obstruction/ Anal Stenosis/ Cow’s Milk Intolerance Starting w/ constipation It is important to appreciate that BO varies from person to person in adults, but even more so in kids, especially when you consider things like BF/ formula, so on and so forth. So for example, BO OD can still be constipated if this is less than their normal, therefore, crucial that this is elucidated. You can see some of the things on screen which are caused by constipation. A whole array of things such as rabbit dropping stools, abnormal retentive posturing and maybe even a palpable stool in the abdo/ PR blood because hard stool difficult to pass and maybe things like pain on passing. Can have things as well like a loss of feeling the need to go etc. All very self explanatory and you pick them up with experience. Overflow important to consider i.e. foul smelling diarrhoea, but actually represents faecal impaction and constipation in the presence of diarrhoea. 14Causes of constipation are a little more diverse than other PX, esp in Paeds. Lifestyle factors are more obvious and don’t require explanation i.e. not drinking enough fluid = harder stools and so on. May have other things like habitually not opening bowels, as this can cause a phenomenon known as rectal desensitization which causes less BO, faecal impaction and loss of feeling of needing to go over time. More organic causes are things such as Hirschsprung’s Disease (will discuss later) and the other things on the screen. Red Flags are important to consider in every Hx, so ensure you screen appropriately. Ask about meconium passage at birth (was it more than 48 hours or delayed? (CF or Hirschsprung’s). Any neuro signs or Sx e.g. SC lesion. Vomiting (e.g. obstructed or Hirschsprung’s). Ribbon stool (anal stenosis) or abnormal anus (IBD, anal stenosis/ sexual abuse). FTT (Coeliac, hypothyroid, safeguarding) Also, worth noting can be idiopathic if no identifiable cause. Tx NICE CKS: - Correct what is reversible - Laxatives (Movicol first line) - Impaction may need disimpaction regime w high dose lax - Praise and encourage toilet (maybe diary, star chart etc – Manchester love this shit). 14 Paediatric Development: Milestones& Delays The next part of the lecture is much more self-directed study based. We will discuss tips and tricks, but unfortunately there is no real way of learning the milestones except for rote learning. 15 Developmental Milestones No easy way of doing this. You need to use the iBook specifically… available on OneMedLearn under Developmental Assessment (as there are differences in what is noted at each age online and you should stick to Manchester’s). These are really boring but the sooner you make notes on it during exam season and not putting it off the better. In the OSCEs… you can be asked to watch a video of a Hx/ child playing during a history and asked about your opinion on it at the end. For this reason, it is crucial you have a good understanding about key milestones and when failure to meet them is considered pathological. Take note (if of nothing else) of the names of each of the 4 domains… Gross Motor, Fine Motor (& vision), Speech language and hearing, then Social and Emotional. These are important to take note of as when you consider if there is a pathology, you must gather whether the issue is in one domain or multiple, which then allows more precise generation of differentials. This is discussed in the following slide. Red flags are most important thing to understand here and are what you should hone in on when making notes, as they are what are alluded to in a developmental milestone Hx/ video (if not normal): Red Flags 16•Lost developmental milestones (REGRESSION) •Cannot hold object at 5 months •Not sitting unsupported at 12 months •Not standing independently at 18 months Not walking independently at 2 years • •Not running at 2.5 years •No words at 18 months •No interest in others at 18 months 16 DevelopmentalDelay GROSS MOTOR DELAY GLOBAL DEVELOPMENTAL DELAY FINE MOTOR DELAY SPEECH/ LANGUAGE DELAY SOCIAL DELAY The 4 domains are really important to understand and everything starts to make sense when you look at Ddx. Global is all domains e.g. Down’s, Fragile X, Fetal Alcohol Gross Motor is only in this domain e.g. C palsy, Spina Bifida, Visual impairment, myopathy Fine motor e.g. dyspraxia, muscular dystrophy, visual impairment Language e.g. social circumstances (multiple lang at home/ siblings who do all talking), hearing impairment, learning diasability, neglect, autism. Need SALT referral, as well as audiology and health visitor… sometimes safeguarding if ?neglect. Personal and Social -> parenting issues, autism, neglect. 17Thankyou!Questions? 18