Normal weight gain in childhood?
Per 3 months
200g/week
150g/week
85-100g/week
50-75g/week
Options for nutritional supplementation?
Concentrate normal formula
High calorie density formula - Infantrini, Nutrini
What causes malnutrition?
Low intake
- physically unable ie. cleft
- anorexia and vomiting
- psychological
Excessive losses
- diarrhoea
- stoma losses
Increased metabolic demand
- cardiac/resp
- recurrent infections
Malabsorption
- coeliac disease
- cystic fibrosis
- enteritis, CMPI
What are indications for gastrostomy/jejunostomy insertion?
- chronic disease with nutritional impairment e.g. CF, BPD
- difficulties with feeding e.g. CP
- GORD with fundoplication
- intensive nutritional therapy e.g. Crohns
Jejunostomy needs continuous feed to avoid dumping
What is obesity and how should it investigated?
BMI >98th centile
TFT, fasting glucose, lipids, insulin level, LFTs
GI manifestations of CF?
Pancreatic
- enzyme deficiency —> steatorrhoea, poor weight gain
- pancreatitis
- insulin dependence
Intestinal
- Meconium ileus
- atresia
- rectal prolapse
- DIOS
- strictures secondary to enzyme replacement
Hepatic
- fatty liver
- cirrhosis
- obstruction of CBD
Comment on jaundice, ascites, portal hypertension
What are colonoscopy findings in Crohn’s disease?
Transmural
Skip lesions
Cobblestone appearance
How does Crohns present?
Abdo pain
Diarrhoea
Weight loss
Can be systemically unwell
Pallor secondary to anaemia
Eyes - uveitis
Joints - arthritis
Skin - erythema nodosum, pyoderma gangrenosum, erythema multiforme
Growth failure, delayed sexual development
How is Crohns investigated?
Bloods
Faecal calprotectin
Colonoscopy and endoscopy
MRI with contrast
How is Crohns treated?
Exclusive enteral nutrition for up to 8w - may need NG Steroids Azathioprine 5-ASA - can use enemas Monoclonal antibodies I.e. info I iamb Abx Surgery
How does coeliac disease present?
Short Pale Reduced subcutaneous fat Poor muscle bulk Buttock wasting Abdo distension
Chronic diarrhoea
Poor weight gain
Irritability
Dermatitis herpetiformis
Which allergens are avoided in coeliac disease?
Gluten
Wheat, rye and barley
Also oats
What is coeliac disease associated with?
Turners Downs T1DM Autoimmune liver conditions Thyroid disease
How is coeliac disease investigated?
IgA total and TGA
If >10x then EMA and no scope
If <10x then scope on gluten
What are histological findings in coeliac disease?
Sub total villous atrophy
Crypt hyperplasia
Inflammation
What are pathological causes of constipation?
Hirschsprungs - bowels opened in first 24hrs
Thyroid disease - ?goitre
Meconium ileus
Spina bifida - comment that lower back normal with normal tone, power, reflexes
Comment that would ideally examine peri anal area
Causes of hepatomegaly?
Infective
- EBV, CMV, hepatitis
Infiltrative
- primary tumour
- secondary e.g. lymphoma, leukaemia, neuroblastoma
Obstructive
- cardiac failure
- hepatic vein thrombosis
Storage disorders (metabolic)
- fat: CF, obesity, malnutrition, TPN
- lipid: Gauchers
- glycogen: glycogen storage disorders
- cholesterol: Niemann Pick
Miscellaneous
- Wilson’s
- alpha 1 antitrypsin
Causes of splenomegaly?
Infective
- acute
- chronic: TB,
- parasite: malaria, toxoplasmosis
Inflammation
- SLE
- JIA
- ITP
Haematological
- leukaemia
- lymphoma
- thalassaemia
- anaemia: sickle cell, spherocytosis, G6PD
Other
- portal hypertension
- Gauchers
- cirrhosis
- cardiac failure
What is portal hypertension and how does it present?
Splenomegaly
Ascites
Portosystemic shunts
FTT
GI haemorrhage
Encephalopathy
Thrombocytopenia, anaemia, leukopenia
What are causes of portal hypertension?
Pre hepatic
- portal vein thrombosis (no liver symptoms)
- umbilical vein catheterisation
- sepsis
Intra-hepatic
- cirrhosis
- congenital hepatic fibrosis
- veno-occlusive disease
Post hepatic
- constrictive pericarditis
- right ventricular failure
- Budd-Chiari (hepatic vein outflow obstruction)
What is congenital hepatic fibrosis?
AR
Look well
Large hard liver, normal function
Large spleen
Large polycystic kidneys
Causes of cirrhosis?
Biliary tract
- Biliary atresia
- Congenital hepatic fibrosis
- CF
- Sclerosing cholangitis
Genetic
- alpha 1 antitrypsin
- Wilsons
- glycogen storage disorder
Infection
- hep B/C
- CMV
Nutrition
- alcohol
- TPN
Causes of ascites?
Infective
- TB
- pancreatitis
Infiltrative
- metastasis
- nephrotic syndrome
- malnutrition
Obstructive
- hepatic vein thrombosis
- cirrhosis
- congestive cardiac failure
How does Alagilles present?
Minimal intra-hepatic bile ducts
Hemivertebrae
Hypoglycaemia
Peripheral pulmonary stenosis
