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Year 4 PAEDS > GI and Liver 3 > Flashcards

GI and Liver 3 Flashcards

1
Q

Summarise bilirubin metabolism

A

Bilirubin is produced as a result of breakdown of haem from haemoglobin
= conjugated bilirubin

Unconjugated bilirubin metabolised by liver cells to a soluble conjugated form and is excreted via the hepatic and bile ducts into the duodenum

Bilirubin and bile salts in the bowel aid absorption of fats and fat-soluble vitamins

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2
Q

What happens to conjugated bilirubin?

A

Approx half of conjugated bilirubin is reabsorbed from the bowel as urobilinogen in the enterohepatic circulation

Urobilinogen may be excreted in urine or remetabolised through the liver

In jaundice - excessive conjugated bilirubin may be excreted in the urine, causing urine to be dark

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3
Q

What are some of the commoner causes of jaundice in childhood?

A 
Mostly unconjugated:
- Haemolytic disease of the newborn
- Sickle cell disease
- Spherocytosis
Rarely:
- Hepatic (rare)
Mostly conjugated
- Hepatitis
- CF
- Cirrhosis
Obstructive:
- Hepatitis
- Biliary atresia
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4
Q

What is important to ask in a history of jaundice?

A

Insidious vs sudden onset - first sign of insidious onset is yellowing of sclera
- haemolysis is usually rapid onset

Malaise
- Duration of malaise, abdo pain, anorexia

Symptoms of anaemia
- Occurs as a result of haemolysis

Pruritus
- Intense skin irritation as a result of bile salts in the skin

Urine colour
- Very dark indicated conjugated bilirubin is the cause

Steatorrhoea

  • Frothy, foul-smelling stool which floats in toilet
  • Commonly seen in children with long-standing cirrhotic liver disease
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5
Q

What may be found on physical examination of a jaundiced child?

A

FTT or poor growth

Skin signs:

  • Scratch marks on skin
  • Signs of long-standing liver disease including spider naevi, clubbing and ascites

Hepatosplenomegaly

  • Hard liver suggests cirrhosis
  • Splengomegaly can suggest haemolysis or cirrhosis
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6
Q

What may bloods reveal in a jaundiced child?

A

Hb:
- Low Hb with increased reticulocytes indicates haemolysis

Bilrubin:

  • Unconjugated = haemolysis
  • Conjugated = hepatic / post-hepatic

Liver enzymes:
- Raised in hepatitis

Alkaline phosphatase:
- Elevated in cirrhosis or in cases of long-standing jaundice

Serology
- Identification of hepatitis virus

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7
Q

What is the most common cause of jaundice in older children?

A

Hepatitis A

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8
Q

What is Reye’s syndrome?

A

Acute encephalopathy associated with fulminant liver failure

Can be induced by aspirin

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9
Q

What metabolic syndromes can lead to progressive jaundice?

A

Wilsons disease

  • Defect in copper metabolism
  • Leads to neurodevelopmental delay and FTT
  • Brown ‘Kayser-Fleischer rings’ around cornear
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10
Q

What enzyme defects can lead to chronic hyperbilirubinaemia?

A

Criggler-Najar disease
- Glucuronyl transferase deficency

Gilbert’s syndrome
- Bilirubin uridine diphosphate glucuronosyltransferase enzyme

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11
Q

What is mesenteric adenitis?

A

Diagnosed where no other cause for acute abdo pain can be found

Caused by acute enlargement of intra-abdo LN as a result of infection in URTI, chest or gastroenteritis

These enlarged LN cause pain

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12
Q

How may mesenteric adenitis present?

A

Recent hx of infection and signs may still be present in chest or throat

Fever, malaise, central abdo pain

NEVER peritonism or guarding = diagnosis of exclusion

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13
Q

What is the management of mesenteric adenitis?

A

Exclude other conditions

Expectant management - usually resolves within 24-48hrs

Analgesia

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14
Q

What is pyloric stenosis?

A

Hypertrophy and hyperplasia of the pylorus muscle

Results in narrowing of the pyloric canal which can then easily become obstructed

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15
Q

When does pyrloric stenosis usually develop? In who?

A

First 4-6 weeks of life

M:F 7:1

Most common in firstborn male children

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16
Q

How does pyloric stenosis present?

A

Projectile vomiting during or immediately after feeding
- But not necessarily at every feed

Infant hungry and ready to take another feed immediately

Vomit may be blood tinged but not bile-stain

Weight loss and dehydration often seen

Constipation

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17
Q

What may be noted on examination of an infant with pyloric stenosis?

A

Visible peristalsis from the LUQ to RUQ immediately after a feed or just prior to vomiting

Hard mobile tumour ‘olive mass’ to R of epigastrium

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18
Q

How should pyloric stenosis be investigated?

A

If olive ass found, no need for barium studies or USS

If diagnosis suspected but tumour not palpable, confirm with USS

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19
Q

What may occur as a result of prolonged vomiting in pyloric stenosis?

A

Loss of acidity from stomach leads to hypochloraemic alkalosis and reduced sodium and potassium levels in serum

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20
Q

What is the management of pyloric stenosis?

A

Surgical treatment

  • Ramstedt procedure involves splitting pyloris muscle without penetrating the mucosa
  • If infant is dehydrated rehydration must take place prior to surgery with replacement of sodium, chloride and potassium
  • Oral feeds can be given within hours postoperatevely
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21
Q

What is the pathophysiology of hypochloraemic, hypokalaemic metabolic alkalosis?

A

Hydrogen chloride loss from vomiting leads to hypochloraemic metabolic alkalosis

Renal compensation by maximising bicarb reabsorption

Exchange of potassium and sodium for hydrogen ions in DCT

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22
Q

What is testicular torsion?

A

More accurately = torsion of the spermatic cord

Common urological emergency

Causes occlusion of testicular blood vessels

Unless rapidly treated leads to ischaemia, leading to loss of the testis

(Germ cells are the most susceptible cell line to ischaemia)

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23
Q

What are the two types of testicular torsion?

A

Intravaginal

Extravaginal

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24
Q

What is intravaginal testicular torsion?

A

Secondary to the lack of normal fixation of the posterior lateral aspect of the testes to the tunica vaginalis

Results in testis being free to swing and rotate within the tunica vaginalis of the scrotum

= “bell-clapper deformity”

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25
Q

What % of testicular torsions are intravaginal and what % of these are bilateral?

A

Intravaginal testicular torsion = 12% males

40% bilateral

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26
Q

What is extravaginal testicular torsion?

A

Occurs more often in neonates and occurs in utero or around the time of birth, before the testis is fixed in the scrotum by the gubernaculum

Thus both the spermatic cord and tunica vaginalis undergo torsion together, either in or just below the inguinal canal

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27
Q

How does testicular torsion present?

A

Sudden, severe pain in one testis

  • Often during sport / physical activity
  • May be associated lower abdo pain

Often hx of a previous similar episode

+/- n&v

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28
Q

Is easing pain a good sign in testicular torsion?

A

Not necessarily - pain also eases as necrosis sets in

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29
Q

What may be found upon examination of testicular torsion?

A

Reddening of scrotal skin

Swollen, tender testis retracted upwards

Lifting testis up over symphysis increases pain
- Unlike epididymitis where this relieves pain

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30
Q

What test can be performed to check for testiclar torsion?

A

Looking for absence of cremasteric reflex

  • 100% sensitivity and 66% specificity for testicular torsion
  • L1/L2 spinal nerves elicited by gentle pinching or stroking of the inner thigh while observing the scrotal contents
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31
Q

Who does testicular torsion typically affect?

A

Neonates and post-pubertal boys

Peak 12yrs

But can affect all ages

32
Q

Which side is more commonly affected by testicular torsion?

A

L more than R

Bilateral rare

33
Q

Ddx testicular torsion (4)

A

Epididymitis, orchitis or epididymo-orchitis
- Usually from reflux of infected urineor from STI

Hydrocele
- Painless and transilluminatable

Incarcerated hernia

Testicular tumour
- Rarely painful

Mumps

  • Swelling of parotid glands
  • Mumps orchitis rare before puberty
34
Q

What investigations are done for testicular torsion?

A

All cases of acute testicular pain are torsion unless proved otherwise
= URGENT TREATMENT

35
Q

What is the management of testicular torsion?

A

Prompt surgical exploration is required to untwist and fix testis to the scrotum

36
Q

What is the prognosis of testicular torsion?

A

If corrected within 6 hours, good prognosis

If >24hrs - may have become necrotic and require removal

The contralateral testis must also be fixed as it is also prone to torsion

37
Q

What is biliary atresia?

A

Important but rare condition

Caused by atresia of intrahepatic or extrahepatic bile ducts

38
Q

How do babies with biliary atresia present?

A

Persistent jaundice with rising conjugated bilirubin

Pale, chalky stools

From 4 weeks of life

39
Q

What happens if biliary atresia goes undiagnosed?

A

Rapid progression to liver failure and death

But if diagnosed within 3 months - surgery can resolve liver function to near normal

40
Q

How is biliary atresia managed?

A

Urgent referral for assessment

Diagnostic isotope scan and surgical correction
- Kasai procedure

41
Q

What is cryptorchidism?

A

Undescended or ectopic testes

42
Q

How can undescended or ectopic testes be differentiated?

A

Only during operation

43
Q

How does cryptorchidism occur?

A

Usually the testes descend from their fetal intra-abdo position through the processus vaginalis and into the scrotum during the 7th month of gestation

The undescended testicle is found along the normal path of descent and the processus vaginalis is usually patent

If bilateral - consider hypopituitarism

Ectopic testicle is one that has completed its path of descent through the inguinal canal but ends up at the wrong destination, usually in the groin

44
Q

How does cryptorchidism present?

A

Asymptomatic

45
Q

What may be found on examination of a child with cryptorchidism?

A

Warm rooms and warm hands

Testes impalpable or high in inguinal region or scrotum

Empty hemi-scrotum

+/- inguinal hernia

Attempt to bring testis down into position by sweeping massage into inguinal region (differentiates from retractile testis)

46
Q

What investigations are performed for cryptorchidism?

A

Clinical diagnosis

USS can help to locate testis

Hormonal testing if testes impalpable bilaterally

47
Q

Who are more likely to be affected by cryptorchidism? How common is it?

A

Premature babies

5% undescended at term
1% by 12 months

(Spontaneous descent may occur in first 6 months but unlikely after this)

48
Q

What often accompanies cryptorchidism?

A

Inguinal hernia

49
Q

What is the management of cryptorchidism?

A

Surgery - ochidoplexy if not spontaneously resolved by one year (most do by 6 months)
- Important to be before age 2 yrs (by this age the number of germ cell s in the undescended is already reduced and the risk of infertility is increased, whether unilateral or bilateral)

50
Q

Other than reduced fertility, what is a risk of delayed surgery in cryptorchidism?

A

Testicular tumour

51
Q

What % of undescended testis are palpable and impalpable? Where are they and how should they be managed?

A

Palpable = 80%

  • Usually in inguinal canal
  • Tests can be bought down into scrotum, referred to paediatric surgeon at one year

Impalpable = 20%

  • Either intra-abdo (risk of malignant degeneration), inside the canal or absent
  • Investigate with laparoscopy
52
Q

What is the most common cause of hepatitis?

A

Viral infections:

  • Hep A (most common)
  • Hep B
  • Hep C (rare)
  • EBV
  • CMV
53
Q

How is Hep A transmitted?

A

Faeco-oral route

Associated with poor hygiene and limited sanitation

54
Q

What age group has the highest incidence of Hep A? What is the incubation period?

A

Age 5-15yr

2-6 weeks

55
Q

How is hep B transmitted and prevented? What is the incubation period?

A

Blood products eg vertical transmission from mother to baby - however if mother has hepatitis B antibodies her baby is protected

Immunisation of the newborn infant is possible if the infected mother is recognised antenatally

2-6 months

56
Q

How does Hep A present?

A

Insidious onset

Child is unwell with anorexia, nausea, and jaundice appears 5 days later

Pale stools and dark urine

57
Q

What may be found on examination of a child with hep A?

A

Tender but not enlarged liver

58
Q

What may be found on investgiation of a child with hep A?

A

LFTS - high transaminases and raised conjugated hyperbilirubinaemia

Identify virus serologically

59
Q

How is hep B serologically investigated?

A

Presence of hep B surface and core antigens

60
Q

How is viral hepatitis managed?

A

No specific treatment

Hep A highly infectious during prodromal phase but by the time jaundice has presented the pt is no longer excreting the virus

Hep B highly contagious

61
Q

What is the prognosis of viral hepatitis?

A

Hep A - good prognosis and rare to develop chronic hepatitis

Hep B - more common to develop chronic hepatitis, and increased risk of developing subsequent hepatocellular carcinoma in adult life for carriers

62
Q

What is Crohn’s disease?

A

Type of IBD that may affect any part of the GI tract from mouth to anus

Discontinuous mucosal involvement

Cobblestoning in severe vases

63
Q

How does Crohn’s disease present?

A 
Recurrent abdo pain
Anorexia
Growth failure
Fever
Diarrhoea
Anaemia
Oral and perianal ulcers
Arthritis
64
Q

How is Crohn’s disease diagnosed?

A

Endoscopic biopsy

65
Q

How is Crohn’s disease managed?

A

Remission induced by:

  • Elemental diet
  • Immunomodulator drugs
  • Anti-TNF drugs eg infliximab
  • Steroids

For localised disease surgical resection may be indicated

66
Q

What is ulcerative colitis?

A

Inflammation with ulcer formation in the lining of the colon

Continuous

67
Q

How does ulcerative colitis present?

A

Bloody diarrhoea
Abdo pain

Possibly:

  • Weight loss
  • Arthritis
  • Liver disturbances
68
Q

What is the management of ulcerative colitis?

A

Mesalazine - PO or PR

Steroid enemas

Severe cases:

  • Immunosuppressive therapy
  • Infliximab
  • Colectomy
69
Q

What is toddlers diarrhoea?

A

Toddlers often experience non-specific diarrhoea probably due to rapid gastrocolic reflex

70
Q

How does toddlers diarrhoea present?

A

Diarrhoea

Drinking excessive fluids esp fruit juices

Food particles in stool

71
Q

How is toddlers diarrhoea diagnosed?

A

Only is child is thriving

72
Q

How is toddlers diarrhoea managed?

A

Reassurance

73
Q

What is a volvulus?

A

Torsion of a mal-rotated intestine

74
Q

How does volvulus present?

A

Severe abdo pain with bilious vomiting

75
Q

How is volvulus treated?

A

Urgent surgery to untwist the volvulus and treat underlying malrotation

If missed bowel may infarct

Year 4 PAEDS (23 decks)

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