Computer generated transcript

Warning!
The following transcript was generated automatically from the content and has not been checked or corrected manually.

FINALS PAEDIATRICS TEACHING SERIES Lecture 1 - Gastroenterology Shruthi Sankaranarayanan Introduction ■ Important to know the key aspects of the list of conditions given in your syllabus – could overlap with adult medicine, so trying to explain some physio so it helps with adult med revision as well ■ Progress test: not about knowing the answer, but about being able to rule out or narrow down from options ■ Aim of this series – to put together the resources available in one place and enable you to start doing questions to remember and consolidate the knowledge ■ Question banks for exam techniqueConditions Covered: Appendicitis Coeliac disease Gastroenteritis Intestinal obstruction Intussusception Hirschsprung’s disease Pyloric stenosis Biliary atresia IBD Wilm’s tumor Abdominal pain Constipation Appendicitis Pathophysiology: • Becomes inflamed due to an infection that gets trapped in the appendix at the point where it links to the colon • When it ruptures à faecal content released into the abdomen à peritonitis à sepsis Sx: • Central abdominal pain that moves into the RIF region; pain over McBurney’s Point : 1/3 the distance between ASIS and umbilicus on the R side • Rovsing’s sign – Pain on the LIF when palpating the RIF • Psoas sign – patient lying on their left side, when R thigh is passively extended, pain on the right side • N&V, not able to move due to the pain • Pain and guarding on palpation of abdomen • Rupture - Rebound tenderness, Percussion tenderness, Haemodynamic instability Ix and important differentials: • Clinical diagnosis + inflammatory markers • USS of pelvis abdomen for differentials • diverticulums– distortion of the distal ileum, hence sx are in the similar region; usually asymptomatic, but can complicate intos volvulus/intussusception – removed prophylactically; Appendix mass – when omentum wraps around the acutely inflamed appendix, causing it to feel like a mass in the RIF region upon palpation à conservative management (as the rupture is contained) with Abx à appendectomy after acute condition resolved; Mesenteric adenitis Mx • Prophylactic Abx IV** • Investigative lapropscopy (if not confirmed) + appendectomy Finals tip:Prior to surgery – IV Abx is given prophylacticallypay attention to the question, next line of treatment would be surgery, but if indicated prior to surgery, rmrbr IV Abx would be considered, NOT other investigations Coeliac Disease - 1 Pathophysiology: • Autoimmune disease with anti-TTG, anti-EMA targeting the villi on the epithelial layer of the small intestine particularly the jejunum à atrophy and subsequent inflammation of villi à malabsorption of nutrients and disease symptoms due to the inflammation particularly to gluten (found inn wheat, barley, rye) • start quite early in childhood, especially in toddlers; but can ideally start at any age; T1DM patients Sx: • GI Disease symptoms – diarrhoea, fatigue/lethargy, abdominal distention, bloating, abdominal pain, etc. • Weight loss AND difficulty growing – failure to thrive • Dermatitis herpetiformis – this is a cutaneous manifestation of Coeliac disease usually on the trunk • Mouth ulcers • Anaemia due to B12 fola,e , oriron deficiency à NEUROLOGICAL SYMPTOMS • peripheral neuropathy, epilepsy, cerebellar ataxia- B12 is needed for nerve cells for formation of the myelin sheathe and to operate properly, hence the deficiency leads to neurological symptoms Ix: • 1 line: Anti-TTG, Anti-EMA, Total IgA - Important to do a total IgA test – if low, test for the Immunoglobulin G version of the same antibodies • 2 line: Endoscopy with a biopsy – crypt hypertrophy (crypts in your intestinal layer) orvillous atrophy and raised intra-epithelial lymphocytes/lymphocytic infiltration Mx: • Life-long gluten-free diet is curative; checking on coeliac antibodies frequently will be helpful in monitoring the disease • Gluten free: maize/corn; rice; potatoes/starch • Gluten-rich: pasta, bread, rye, oats, barley, etc. • Adult patients with coeliac disease should receive pneumococcal vaccine for every 5 years as coeliac disease puts them at risk of hyposplenism that causes pneumococcal infection to affect them worse than others Coeliac Disease – 2 Complications: • usually due to lack of diagnosis and hence untreated coeliac disease • Malnutrition: Vitamin deficiency, osteoporosis • Inflammation: ulcerative jejunitis • Cancer: small bowel adenocarcinoma non-,odgkin lymphoma enteropat,y-associated T-cell lymphoma Finals tips: • T1DM diagnosis à test for anti TTG and anti EMA • A lot of autoimmune conditions are associated with coeliac: Down’s, thyroid disease, PSC, PBCirrhosis, Autoimmune hepatitis • When investigating , important that the patient is ON THEIR NORMAL GLUTEN DIET for atleast 6 weeks prior to testing as otherwise, it will not be possible to find the inflammation or antibodies needed to diagnose them with coeliac • They tend to like to test symptoms of coeliac in older patients with a slant towards what type of cancer they could get • Tend to present as paeds patients with problems growing or dropping on the weight and height percentile Gastroenteritis - 1 Pathophysiology: • Viral - Abdo pain, diarrhoea/loose stools; highly contagious, especially between family members; Rota virus, Nora virus, Adenovirus (uncommon) • Bacterial – based on the causative pathogen; abdo pain, usually bloody diarrhoea, cramping, fever , sometimes vomiting; CRUX of most qns is identifying the pathogen; MUST do a stool sample and isolate organism if antibiotic Tx is considered E.coli Shigella Campylobacter Jejuni Salmonnella Bacillus Cereus Yersinia Enterocolitica Staph. Aureus • Most common • Similar to E.coli as • Traveller’s • spread by eating • grows on cooked • spread by eating • characterised by cause of produces shiga diarrhoea raw eggs/poultry food that is not undercooked perfuse vomiting traveller’s toxins– hence, can • spread by immediately pork/raw pork apart from the diarrhoea cause HUS as well uncooked poultry, refrigerated/partial • longer toshow other symptoms (>campylobacter • Mx: Abx can also unpasteurised milk, cooked symptoms and • similar timingas jejuni) increase HUS risk untreated water; • Exam favourite: longer >2 weeks to Bacillus Cereus • produces Shiga so DO NOT GIVE • incubation period is patient eats fried resolve • non-bloody toxins unless severely 2-5 days and rice left at room • Think: Y-lower in the diarrhoea • damages blood compromised resolvesafter 3-6 temp. à vomiting alphabet = longest cells and can days 5hrs after à to resolve cause HUS (type • Mx: Abx can be diarrhoea 8 hours • Right-sided of intrarenal considered in after à resolves abdominal pain as cause of AKI severe symptoms after 24 hours this pathogen can where tiny blood and other • the immediate toxin cause mesenteric clots are formed immunocompromisi release - early adenitis in the renal blood ng RFs like HIV, HF, vomiting; • Mx: Usually self- vessel) etc. – • it breaks down into resolving • Mx: Abx can also other toxins in the increase HUS risk intestines - watery DO NOT GIVE so diarrhoea unless severely • Think B: highest in compromised the alphabet = fastest to resolve Gastroenteritis – 2 Mx: 1. Infection control and barrier nursing • put in a separate bay to control infection • Children must not attend school atleast for 48 hours after all symptoms have resolved 2. Preventing dehydration and providing rehydration • If tolerating oral intake à oral fluid challenge, usually a type of Oral Rehydration Solution (ORS) like dioralyte can be used every 5-10 mins • If clinically dehydrated and not tolerating oral intake: IV fluids • Slowly reintroduce solid foods like toast 3. Antibiotics – must do stool sample ad isolate pathogen 4. Do NOT give anti-diarhhoeals like loperamide or anto-emetics like metoclopramide or cyclizine Complications: • Transient lactose-intolerance post gastroenteritis • Guillain-barre syndrome – ascending neuropathy 2° to an infection • IBS The questions on passmed to do with testing which organism causes it tend to be a little misleading – so if you get them wrong, I would not worry – just learn the above facts Intestinal obstruction Common causes: • Meconium ileus – infant/neonate does not pass stools within 48 hrs due to an obstruction • Hirschsprung’s disease • Duodenal /oesophageal atresia - congenital malformation of either part • Volvulus or Intussusception • Strangulated hernia Presentation: • Complete constipation – inability to pass stools or wind • Abdominal distention and colicky abdominal pain • Auscultation – no bowel sounds or ‘high-pitched tinkling sounds’ • Vomiting – bilious; bright green (note: if non-bilious, think cause is higher up the GI track) Ix: • AXR - will show dilated loops of bowel Complications and Mx: • Emergency! Intestinal obstruction can perforate and cause sepsis • Initial Mx:Make NBM and put on NG tube to drain stomach of contents and replenish electrolytes and fluids • Surgical intervention – usually paediatric surgery unit; sometimes teenagers can be managed by adult surgical units provided there is a paeds department in the hospital Intussusception Pathophysiology: • The bowel telescopes/loops into itself or invaginates into itself à thickening of the bowel wall AND narrowing of the bowel lumen; almost always ileo-caecal region • The section of the bowel that is trapped is devoid of blood supply à ischaemia à mucosa responds by sloughing into the gut (stools with blood + mucus) • Usually on a background of a recent viral URTI Sx: • Constipation and severe colicky abdominal pain • Red-currant jelly stools • Palpation shows ‘sausage-shaped’ mass • Pale, lethargic, unwell child Ix and Mx: • USS to confirm • Therapeutic enema – forceful pumping of contrast, air or water pushes the bowel back to normal Complications: • Gangrenous bowel due to severe ischaemia – perforation à sepsis Hirschsprung’s Disease Pathophysiology: • Congenital condition due to incomplete development of the myenteric plexus/Auerbach’s plexus - enteric nervous system/ ’brain’ of the GI trac– initiates and controls peristalsis • Parasympathetic ganglion cells start developing from the mouth and move down GI track à development stops prematurely before reaching distal colon or rectum (aganglionosis) à lack of relaxation of the colon à constant constriction à obstruction à Hirschsprung’s disease • Severity of disease and age of presentation depends on when the development stopped • Associations with Downs syndrome, MENS II, NFM; more common in boys Sx: • Classically: infant - meconium passed 48 hours post-birth , distended abdomen, bilious vomiting • Classically: toddler –hronic constipation since birth , failure to thrive, distended abdomen, vomiting • PR exam causes explosive diarrhoea • Hirschsprung-Associated-Enterocolitis (HAEC) – inflammation due to C.Diff; baby presents very poorly and septic Ix: • AXR – to confirm abdominal distention • GOLD STANDARD: Rectal biopsy to determine colonic aganglionosis Mx and Complications: • HAEC – Fluid resuscitation, IV Abx, sepsis management • Pre-surgery – rectal washout/irrigation to prevent C.diff • Surgery to remove the non-innervated part of the colon, and attach the normal region to the anus – usually good prognosis, but can have long-term implications like faecal incontinence, or enterocolitis post-surgery due to change in gut flora Pyloric Stenosis Pathophysiology: • Hypertrophy of the smooth muscle of the pyloric sphincter à constriction of canal à partial obstruction causes the force of peristalsis to increase à forceful ejection à projectile vomiting • Polygenic inheritance pattern – females are more likely to pass on to offspring as they become adults Sx: • Classically young baby with failure to thrive – not gaining weight, not keeping fluids or feeds in for couple days to a week • Projectile or forceful vomiting that isnot bile-lined – IMPORTANT: non-bilious because the food has not gone down the small intestine yet, hence, no interaction with bile just yet • ‘Olive-shaped mass’ in the upper part of the abdomen – hypertrophic pyloric sphincter Ix: • USS abdomen is diagnostic • Generally clinical diagnosis in GP à sent into hospital for USS and surgery • Electrolyte studies – Hyochloraemic, hypokalaemic, metabolic alkalosis on ABG • Hyperchloraemia and metabolic alkalosis – due to loss of HCl directly from stomach • Hypokalemia – kidneys retain H+ ions as compensation in return for K+ ions Mx: • Ramstedt’s operation – incision on the smooth muscle layer of the pyloric sphincter to release obstruction and widen canal Biliary atresia - 1 Pathophysiology: • Congenital malformation of bile duct à build-up of conjugated bilirubin in the blood à Prolonged jaundice in newborns/infants • Physiological/harmless Jaundice in newborns or infant: high RBC content that is slowly being excreted (esp. in premies), or feeding breast milk à build-up of unconjugated bilirubin • à phototherapy being used to get rid of this directly • à transported to the liver à conjugated bilirubin • à that exits via the bile duct to SI to be reabsorbed into the liver via portal circulation -enterohepatic circulation of bile • à or still exits via bile duct to SI to becreted as stools , or to the kidneys to beexcreted via urine • Conjugated bilirubin is built updue to damaged bile duct à difficult to drain the conjugated bile into the SI à pathological jaundice Biliary Atresia - 2 Sx: • Classically parents coming in with baby not tolerating feeds, looking unwell/yellow, pale stools (lack of stercobilin) • Jaundice for more than 14 days in normal babies and 21 days in pre-term babies • Hepatomegaly or splenomegaly Ix: -Reason for Ix apart from diagnosing is also ruling out other important differentials of this presentation • Conjugated bilirubin levels: GOLD standard, if high • Alpha-antitrypsin 1 levels – deficiency of this is a common cause for increased conjugated bilirubin levels • Heel-prick test – to test forCystic fibrosis which can cause problems with bile duct obstruction • Protein electrophoresis – tests for other liver diseases Mx: • First-line:asai portoenterostomy – bile duct attached directly to SI; not curative, only treats jaundice • Second-line ad cursative: Liver transplant • Adjuvant therapy: Ursedeoxycholic acid – used post-surgery to facilitate weight gain, improve bile movement IBD – 1 Pathophysiology: • Quite prevalent in children and management is similar as adults • UC: adults – tends to affect distal colon; Paeds: pancolitis so removal of the entire colon and rectum is usually curative • Can be quite non-specific presentation in children – growth failure ,ate puberty S, similar to anorexia nervosa , abdominal pain, etc. Sx: • Diarrhoea • Bleeding and anaemia • Weight loss • Dermatological manifestation – erythema nodosum pyode,ma gangrenosum , clubbing, scleritis, episcleritis • Flares: systemically unwell – fever, dehydration, lethargy, etc Ix: • Blood tests – Hb, TFTs, CRP, ESR, U&Es, LFTs, etc. • Faecal calprotectin • Imaging: Endoscopy (OGD and colonoscopy) + biopsy – gold standard • USS, CT, MRI can be used for complications, especially with Crohn’s – fistulae, abscesses, strictures, etc. IBD – 2 UC: Crohn’s: Sx and Path: Sx and Path: • CLASPE • Crow’s NESTS C • ontinuous inflammation • N o blood or mucus (less common) • Limited to colon and rectum usually; adults – distal colon, • E ntire GI tract paeds – pancolitis • S kip lesions on endoscopy • Aminosalicylates Tx • Superficial mucosa affected • Terminal ileum is most affected; Transmural impact of intestines • Primary sclerosing cholangitis (PSC) • S limmer – weight loss; Smoking is a risk • Excrete blood and mucus in stools factor Mx: Mx: Acute episode needing inducing of remission for severe condition: Acute episode needing inducing of remission: • First line: IV Hydrocortisone or Oral Pred • First line: IV Hydrocortisone or Oral Pred • Second line: IV ciclosporinn • Second line: ADD immunosuppressants – azathioprine/mercaptopurine (1 st Acute episode needing inducing of remission for mild-moderate condition: line)/methotrexate/infliximab/adalimumab • First line: Aminosalicylate like mesalazine/balsalazide • Second line: Corticosteroids oral pred Maintaining remission: • First line:Azathioprine or Mercaptopurine or Methotraxate or Infliximab or Adalimumab Maintaining remission: • Second line: Surgery – only when the affected area • First line: Aminosalicylate is the terminal ileum; sometimes also used to • Second line: Azathioprine/mercaptopurine surgically remove strictures, fistulae, etc. • Surgery: generally affects pancolon in kids – panprotocolectomy with a ileostomy or ileo-anal J pouch: the ileum is folded on itself, motion and this is attached to the anusols before it passes as a Wilms’ tumor Pathophysiology: • The most common solid paediatric tumors: Brain tumors, neuroblastoma, nephroblastoma – Wilm’s, rhabdomyosarcoma Sx: • childrenlunder 5 years of agenless abdominal mass 1/2,episodes of haematuria , and slightly elevated blood pressure in • Sometimes they can present with abdo pain, fever, lethargy, etc. – but mostly asymptomatic presentation • Wilms is associated with other syndromes – can present with macroglossia, horizontal earlobe crease (Frank’s crease Ix • USS Abdo to visualise tumor à CT to stage à Biopsy to confirm the histology of tumor as a possible differential is neuroblastoma • Mets tend to be in the lungs – it tends to be asymptomatic – CT chest and CXR is required to check for mets Mx: • Nephrectomy and surgical excision of the tumor + Adjuvant chemo or radio therapy • If mets – poorer prognosis Finals Tips: • Consider Wilms tumor in all children that present with a painless abdominal mass – DO A URINALYSIS • neuroblastoma must be consideredl mass wdifferentiated by: elevated blood pressure – while possibly Wilm’s, • USS of abdomen will show the adrenal or renal mass WITH CALCIFICATION in neuroblastoma • Mets can be in bones, nephroblastoma (Wilms) tend to be localised, if mets – commonly in lungs1. Stool sample Question 1: 2 year old boy presents to GP 2. AXR with his mother who is 3. Hydrogen breath test worried that he is ot growing 4. IgA TTG antibodies as fast as other children in his 5. Abdominal USS pre-school. She also described smelly diarrhoea multiple times a week and that he also compainns of 4. IgA TTG antibodies abdominal pain. O/E: He has He is presenting with classical coeliac a bloated abdomen, wasting disease presentation. in his glutes, and he has dropped 2 centile lines and falls on the 10 centile. Which investigation will reveal the diagnosis?1. Faecal calprotectin Question 2: 2. FIT test – faecal immunonchemical test 10 year old girl with bloating, diarrhoea and severe tummy 3. Duodenal biopsy pain for 5 months is seen in the 4. Serum IgA tTGA and total IgA gastroenterology clinic. 5. Serum IgA EMA and total IgA Investigations are run, and the following are the results of the FBC: 5. Serum IgA EMA and total IgA Anaemia + diarrhoea + bloating = coeliac Hb – 120g/L (M: 135-180 F: 115-160) Platelets – 200*10^9/L (150-400) WBC 8*10^9/L (4.0-11.0) What is the most appropriate next investigation?1. Abdominal pain Question 3: 2. Bloody diarrhoea A 14-years old boy presents with 3. Mouth ulcers GI symptoms to the 4. Perianal malformations i.e. skin tags gastroenterology clinic and a 5. Persistent flu-like symptoms diagnosis of Crohn’s is considered. What is the most common symptom of Crohn’s in children? 1. Abdominal Pain Non-specific GI symptoms + delayed development of puberty is a more common presentation of crohn’s in children compared to adults who present commonly with bloody diarrhoea1. Sepsis Question 4: 2. Rotavirus 9 year old girl presents to ED 3. Intestinal obstruction with 24hours of N&V. She has 4. Bacillus cereus now developed abdominal pain 5. Diabetic ketoacidosis and is also noted to have deep and laboured breathing. This is new as she is otherwise fit and well without any other 5. Diabetic ketoacidosis prescribed medication. These Tricky one! Sounds like possible are her blood test results: gastroenteritis but the laboured breathing Na+ 130mmol/L (135-145) and metabolic acidosis points at DKA K+ 4.5mmol/L (3.5-5.0) HCO3- 14mmol/L (22-28) QUESTIONS & FEEDBACK https://docs.google.com/forms/d/ rm_m5ULj4u9MJd_vVazy0XXpgS0w hA/viewform?usp=sf_link