Paediatrics Flashcards

1
Q

What is biliary atresia

A

= a congenital condition where a section of the bile duct is either narrowed or absent.

  • There is progressive inflammation & fibrosis of the extrahepatic ducts.
  • This leads to cholestasis, liver damage, and liver failure.
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2
Q

How does biliary atresia present?

A

History:

  • Symptoms may not present until after the first week of life.
  • Persistent neonatal jaundice (>14days in term, formula fed, >21days in premature, breast fed)
  • Pale stools
  • Dark urine

Examination:

  • Jaundice
  • Findings in >3 month old:
    • Hepatosplenomegaly
    • Ascites
    • Failure to thrive →malabsorption of fats
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3
Q

What investigations are done for suspected biliary atresia?

A

Laboratory:

  • Newborn blood spot screening → rules of CF
  • LFTs → raised conjugated bilirubin, raised GGT
    • Physiological jaundice would cause an unconjugated hyperbilirubinaemia

Imaging:

  • Ultrasound → first line
  • Percutaneous liver biopsy → preferred diagnostic investigation
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4
Q

How is biliary atresia managed?

A

Surgical Management:

  • Kasai portoenterostomy → removal of the damaged bile ducts & replacement with a loop of intestine to allow bile to flow from the liver to the intestine.
  • Not curative, usually the child will need a liver transplant at some point.
  • Do within the first 45 days of life.

Liver transplant:

  • If surgery unsuccessful, child is failing to thrive, end-stage liver failure
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5
Q

How does pyloric stenosis present?

A

Presents within the first few weeks of life.

  • Projectile vomiting → non-bilious
  • Weight loss/failure to thrive
  • Constipation
  • Lethargy
  • Firm, round mass (feels like an olive) can sometimes be felt in the upper abdomen due to the hypertrophic muscle of the pylorus.
  • Visible gastric peristalsis
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6
Q

How is pyloric stenosis diagnosed & managed?

A

Diagnosis is made using an abdominal ultrasound.

Laparoscopic pyloromyotomy → an incision is made in the smooth muscle of the pylorus to widen the canal allowing the food to pass from the stomach to the duodenum as normal.

  • Excellent prognosis with this.
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7
Q

What are the risk factors for GORD in babies?

A
  • Premature birth → key
  • Parental history of GORD
  • Obesity
  • Hiatus hernia
  • Cerebral palsy
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8
Q

How does GORD present in children?

A

If <1yrs old:

  • Effortless posseting
  • Early onset (within first few weeks)
  • Time taken to feed >30mins
  • Distressed behaviour during meal times → crying while feeding, refusing to feed
  • Hoarseness and/or chronic cough
  • Faltering growth

If >1yr old:

  • Retrosternal pain
  • Epigastric pain
  • Nocturnal cough
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9
Q

How is GORD managed in children?

A

Supportive:

  • Small, frequent meals
  • Burping regularly to help milk settle
  • Not over-feeding
  • Keep baby upright after feeding
  • Tilt cot mattress

If more problematic:

  • Gaviscon mixed with feeds - can be constipating
  • Thickened milk or formula
  • PPIs (omeprazole)
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10
Q

What are some red flags of constipation in childhood?

A
  • Not passing meconium with 48hrs of birth → CF, Hirschsprung’s disease
  • Neurological signs of lower limbs → cerebral palsy, spinal cord lesion
  • Vomiting → intestinal obstruction, Hirschsprung’s disease
  • Ribbon stool → anal stenosis
  • Abnormal anus → anal stenosis, IBD, sexual abuse
  • Failure to thrive → coeliac disease, hypothyroidism, safeguarding issue
  • Abnormal lower back or buttocks → spina bifida, spinal cord lesion, sacral agenesis
  • Acute severe abdominal pain & bloating → obstruction or intussusception
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11
Q

How is childhood constipation managed?

A
  • Treatment of constipation can be a prolonged process, potentially taking months
  • Correct any reversible contributing factors → high fibre diet & good hydration important
    • Milk is constipating, reduce this & increase water intake.
  • Start laxatives → movicol first line, lactulose second line (stool softeners)
    • Continue long term, slowly wean off as the child develops a regular bowel habit.
  • Faecal impactation may require a disimpaction regimen → high doses of laxatives at first
  • Encourage & praise child visiting the toilet → scheduling visits (sit them on the toilet 30mins after a meal), a bowel diary, star charts
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12
Q

What is Cows Milk Protein Allergy? What are the two types?

A

= A hypersensitivity to the protein in cow’s milk, which can be IgE or non-IgE mediated.

  • IgE mediated → rapid reaction to cow’s milk (occuring within 2hrs of ingestion)
  • Non-IgE mediated → reactions occur slowly over several days.
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13
Q

How does Cows Milk Protein Allergy present?

A
  • Effortless posseting
  • Blood in stool
  • Abdominal pain
  • Diarrhoea
  • Urticarial rash (hives)
  • Angio-oedema
  • Cough or wheeze
  • Sneezing
  • Watery eyes
  • Eczema, dry skin
  • Anaphylaxis → only in severe cases.
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14
Q

How is Cows Milk Protein Allergy managed?

A

Avoiding cow’s milk should fully resolve symptoms, but it will take several weeks to do this washout:

  • Breast feeding → mothers should avoid dairy products
  • Formula → replace with hydrolysed formulas (protein is broken down)

Most children will outgrow CMPA by 3 years old, often earlier.

Every 6 months, children can be tried on the first step of the milk ladder, and then slowly progress up the ladder until they develop symptoms.

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15
Q

What age is intussusception most common?

A

Most common in children aged 6 months - 2 years.

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16
Q

What causes intussusception?

A
  • No clear cause in most cases
  • May be associated with preceding/concurrent viral infection
  • Pathological lead points → an abnormal area of bowel which is caught & pulled by peristalsis.
    • Meckel diverticulum
    • Intestinal polyps
    • Cystic fibrosis
  • Henoch-Schonlein Purpura
17
Q

How does intussception present?

A

Typical triad of symptoms:

  • Severe, colicky abdominal pain → intermittent, episodes where the child is inconsolable & draws their knees up to their chest
  • Vomiting → becomes bilious in later stages when bowel obstruction occurs
  • Redcurrent jelly stool → a late feature that occurs when ischaemic mucosal tissue is sloughed off & excreted in the stool mixed with blood & mucus.

Other symptoms:

  • Pale, lethargic, unwell child → worsens as dehydration worsens.
  • RUQ mass on palpation, sausage-shaped
18
Q

What investigation is gold-standard in suspected intussusception, and what does it show?

A

Abdominal USS → gold standard
- Shows a target sign

19
Q

How is intussusception managed?

A

Acute Management:

  • Prompt fluid resuscitation → failure to do so is one of the main causes of mortality
  • Analgesia
  • Insertion of NG tube to decompress the stomach
  • NBM

Definitive Management:

  • Non-Surgical → therapeutic enemas are first line
    • Contrast, air, or water are pumped into the colon through a foley catheter to force the folded bowel out of the bowel & into normal position.
    • Contraindicated if there is evidence of shock, perforation, or peritonitis.
  • Surgical
    • Where enemas are unsuccessful or contraindicated, to manually reduce the intussusception.
    • If the bowel become gangrenous or the bowel is perforated, then surgical resection is required.
20
Q

How does appendicitis present?

A
  • Abdominal pain
    • Central initially, moves down to the RIF within the first 24hrs & remains localised here.
    • Tenderness at McBurney’s point (1/3 of distance from ASIS to umbilicus)
    • Rebound tenderness in RIF → increased pain when suddenly releasing the pressure of deep palpation.
      • Indicate peritonitis
  • Nausea & vomiting
  • Low-grade fever
  • Anorexia
  • Rovsing’s sign → palpation of the LIF causes pain in the RIF
21
Q

If not detected on the heel-prick test, how may a child present with cystic fibrosis?

A
  • Meconium ileus - not passing meconium within 24hrs, abdominal distention, vomiting
  • Recurrent LRTIs, chronic cough, thick sputum production
  • Failure to thrive
  • Pancreatitis
  • Steatorrhea
  • Abdominal pain & bloating
22
Q

What is the gold standard investigation for cystic fibrosis?

A

Sweat test -> will show a raised chloride concentration

23
Q

What respiratory management is given to people with CF?

A
  • Chest physiotherapy - to help clear mucus
  • Antibiotics
    • Prophylactic (flucloxacillin) & treatment
    • Oral, nebulised, intravenous
  • Mucolytic drugs
    • DNAse - breaks down the DNA in neutrophils which makes the mucus less viscous
    • Hypertonic saline - helps to rehydrate the mucus - makes it easier to clear.
  • Vaccinations - pneumococcal, influenza, varicella
  • Lung transplant - not many are done
24
Q

What GI management is given to people with CF?

A
  • High calorie diet
    • For malabsorption, increased respiratory effort, coughing, and infections
  • Creon tablets
    • To digest fats in patients with exocrine insufficiency
  • Liver transplant - if failure
25
Q

How does CF affect the respiratory system? What are the clinical complications of this?

A
  • Thickened secretions reduce mucociliary clearance from bronchi & increase salt concentration leads to impaired bacterial defences.
    • Bacterial colonisation & lung inflammation increases.
    • Causes a repeated cycle of infections causing sticky mucous & vice-versa → leads to bronchiectasis.
  • Thickened secretions in sinuses lead to recurrent infection & subsequent nasal polyps due to chronic inflammation.
  • Respiratory complications:
    • Recurrent infections
    • Bronchiectasis
    • Respiratory failure
    • Pneumothorax
    • Haemoptysis
    • Nasal polyps
    • Chronic sinusitis
    • Allergic bronchopulmonary aspergillosis (occasionally seen in asthma, but mostly a CF complication)
26
Q

How does CF affect the male fertility system?

A
  • Thick secretions during development lead to Congenital Bilateral Absence of the Vas Deferens
  • Sperm is produced, but cannot reach the semen, so fertility treatments can support people with CF having children.
27
Q

How is DKA managed in children?

A
  • Correct dehydration
    • Correct evenly over 48hrs to reduce the risk of cerebral oedema
    • Only give a fluid bolus if severe
    • Add potassium & monitor closely - due to prior lack of insulin (which normally drives K+ into cells), treatment causes the risk of hypokalaemia.
  • Fixed-rate insulin transfusion
    • Add IV dextrose once blood glucose falls below 14mmol/L
  • Antibiotics if underlying infection
28
Q

What is the criteria for a diagnosis of DKA?

A

To diagnose DKA you require:

  • Hyperglycaemia(blood glucose > 11 mmol/l)
  • Ketosis(blood ketones > 3 mmol/l)
  • Acidosis(pH < 7.3)
29
Q

What fluids are given in paediatrics for maintenance, replacement, and resuscitation?

A

Maintenance & replacement -> 0.9% NaCl + 4% glucose

Resus -> 0.9% NaCl

30
Q

How is the volume of maintenance fluids in children determined?

A

Based on the child’s weight.
First 10kg weight - 100ml/kg/day
Second 10kg - 50ml/kg/d
For every kg above 20kg - 20ml/k/day

31
Q

How is a fluid deficit & total fluid requirement calculated in paediatric replacement fluid prescribing?

A
  • Clinical signs of dehydration are only detectable when the patient is 2.5-5% dehydrated.
    • Therefore if the child has symptoms/signs of dehydration, but no red flags → 5% dehydrated
    • If any red flags of dehydration or child is clinically shocked → 10% dehydration. If shocked, treat as below.
  • Calculating a fluid deficit → % dehydration x weight (kg) x 10
  • Total fluid requirement → maintenance fluids + fluid deficit
32
Q

What volume of resuscitation fluid is given in paediatrics if the patient is shocked?

A

A standard bolus of 10mL/kg over 10 minutes.

33
Q

What is Henoch-Schonlein Purpura & how does it present?

A

= an IgA vasculitis that presents with a purpuric rash affecting the lower limbs & buttocks in children.

  • Inflammation occurs due to IgA deposits in the blood vessels of affected organs → skin, kidneys, GI tract.

History:

  • Purpuric rash
    • Due to inflammation & leaking of blood from small blood vessels under the skin, forming purpura.
    • Typically purple & palpable under the skin.
    • Typically start on the legs & spread to buttocks → can also affect the trunk & arms.
  • Abdominal pain
    • Associated with nausea & vomiting
    • Bloody diarrhoea may be seen
  • Joint pain
    • Hips, knees & ankles → can be associated with swelling
  • Kidney involvement
    • Frothy urine
    • Haematuria
  • Fever → low-grade
  • Preceding upper respiratory tract infection/gastroenteritis
34
Q

How is Henoch-Schonlein Purpura managed?

A

Most children with HSP have a complete spontaneous recovery within weeks to months, but may require supportive care:

  • Analgesia → paracetamol
  • Rest & good hydration
  • Prednisolone → for severe pain

Follow up for renal involvement & blood pressure is important