Conditions Part 4

Question 1

What is malabsorption in a Child?

Answer 1

Lactose Intolerance

Lactase deficiency (lactose → glucose and galactose) -> lactose ferments in gut -> ↑ waste gas -> pain and bloating

affects 75% of world population - asian, African and hispanic

1 - lactase deficiency
2 - damage to gut, lactase deficiency - gastroenteritis, Crohn’s, coeliac, alcoholism

Question 2

Signs and symptoms of malabsorption?

Answer 2

wind, diarrhoea, bloating with lactose ingestion, abdominal rumblings and pain

Question 3

Investigations for malabsorption?

Answer 3

Clinical diagnosis
Breath Hydrogen Test - GIT bacteria, extend to small intestine from large intestine
Lactose Intolerance Test
FBC - to rule out secondary disease

Question 4

Management of malabsorption?

Answer 4

Dietician referral

o Avoid milk and dairy products -> provide calcium and vitamin-D supplementation

Question 5

What is Coeliac Disease?

Answer 5

Autoimmunity to gliadin (in gluten, wheat, barley and rye) -> shorter villi and flat mucosa

• damage to proximal small intestinal mucosa
• common in 1% of infants
• HLA DQ2 + DQ8

Signs - failure to thrive, abdominal distension, bloating, irritability, dermatitis herpetiformis - knee rash

Question 6

Investigations of coeliac disease?

Answer 6

Serology - IgA tissue transglutaminase

FBC and blood smear - iron def, vitamin B12/ folate, vitamin D deficient

Question 7

Management of coeliac disease?

Answer 7

remove wheat, rye, barley products

MDT - dietician, child psychologist, school involvement, GP, paediatric gastroenterologist

Dietician referral

Question 8

What is Mesenteric Adenitis?

Answer 8

Mainly in children <15yo; recent viral/bacterial infection -> common cause of abdominal pain

abdominal pain - central or RIF
nausea + diarrhoea
decreased appetite
high temperature, lymphadenopathy
increased WCC

Question 9

What are the investigations for mesenteric adenitis?

Answer 9

Large mesenteric lymph nodes seen at laparoscopy (w/ normal appendix) -> definitive

Question 10

What is the management for mesenteric adenitis?

Answer 10

simple analgesia
antibiotics
safety net for increased pain and deterioration

Question 11

What is a hernia? What are the types?

Answer 11

indirect inguinal, umbilical, epigastric, femoral

Question 12

Describe features of an Indirect Inguinal Hernia?

Answer 12

testicle creates a passage (process vagialis) as it travels into the scrotum
failure of passage to close - abdominal lining and bowel protrude through defect

risk factors - male, prematurity, connective tissue disorders

Signs - scrotal sac enlarged, palpable bowel loops, swelling or bulge

Investigations - clinical diagnosis, more common on the right testicle

Question 13

How to manage an indirect inguinal hernia?

Answer 13

Urgent Surgical correction (lap or open) = Elective herniorrhaphy (risk of strangulation/incarceration)

· <6w old correct within 2 days

· <6m old correct within 2 weeks

· <6yo correct within 2 months

Question 14

What is an umbilical hernia?

Answer 14

common in new borns and often resolve
< 1 yo -> watch and wait
>1 -> large and symptomatic = surigcal repair at 2-3 , small/asymptomatic - surgery 4-5 years

Question 15

What is a femoral hernia?

Answer 15

difficult to differentiate from indirect
located between inguinal canal
differentiation often made during operation

Question 16

What is gastroschisis?

Answer 16

paraumbilical abdominal wall defect > abdominal contents outside body, without peritoneal covering

o Manage with immediate surgery (cover with cling-film)

Question 17

What is omphalocele/exomphalos?

Answer 17

bowel protruding out the body with a peritoneal covering / umbilical attached

o Manage with staged closure starting immediately, finishing at 6-12 months

o Chromosomal abnormalities in 15% of cases (Trisomy 13 (Patau’s), 18 (Edward’s), 21 (Down’s); Turner’s)

Question 18

What is Encopresis?

Answer 18

soiling of underwear with stool in children who are past the age of toilet training (>4yo)

o Usually due to constipation with overflow

Question 19

Management of Encopresis?

Answer 19

enquire about psych stressors, changes in medications, food intolerances, etc

Question 20

What is Acute Liver Failure?

Answer 20

massive hepatic necrosis with loss of liver function ± hepatic encephalopathy

o Uncommon; high mortality

o Majority from paracetamol overdose, infection and metabolic disease

Question 21

Causes of acute liver failure in children under 2

Answer 21

infection
metabolic disease
seronegative hepatitis
drug induced
neonatal haemochromatosis

Question 22

causes of acute liver failure in children over 2?

Answer 22

Paracetamol overdose
mitochondrial disease
seronegative hepatitis 
wilson's disease
autoimmune hepatitis

Question 23

Causes of acute liver failure in older children?

Answer 23

viral infection
autoimmune hepatitis
non-alcoholic fatty liver disease
drug induced
Wilsons disease

Question 24

Signs and symptoms of Acute liver failure?

Answer 24

Jaundice

Encephalopathy (alternative irritable -> confusion/drowsiness episodes)

Coagulopathy

Hypoglycaemia

Electrolyte disturbance

Older children (aggressive, unusually difficult)

Question 25

Investigations of Acute Liver failure?

Answer 25

LFTs - AST/ALT high, ALP HIGH, 
Liver function - INR
Liver Inflammation - AST adn ALT
clotting abnormal 
plasma ammonia - rasied 
EEG adn CT - Acute hepatic encephalopathy

Question 26

management of acute liver failure?

Answer 26

refer to national paediatric centre
stabilise the child:
maintain blood glucose
preventing sepsis - antibiotics + antifungals
preventing haemorrhage - iv vitamin K
prevent cerebral oedema - fluid restriction and mannitol

Question 27

Complications of acute liver failure

Answer 27

hepatic encephalopathy

cirrhosis or portal hypertension

Question 28

Management of autoimmune hepatitis?

Answer 28

Prednisolone and azathioprine
sclerosing cholangitis - ursodeoxycholic acid - aids bile flow
liver transplants

Question 29

Management of Wilson’s disease?

Answer 29

Zinc
Pencillamine
Pyridoxine 
Symptomatic treatment for tremor, dystonia and speech impediment 
liver transplantation

Question 30

Management of Non-Alcoholic Fatty Liver Disease?

Answer 30

Weight loss
Statins
Ursodeoxycholic acid - improved bile flow
Treatment of insulin resistance and diabetes
vitamin E + C