what is Meckels diverticulum
Meckel’s diverticulum is a congenital remnant of the vitellointestinal duct, a connection between the embryonic gastrointestinal tract and the yolk sac.
how can Meckels diverticulum present?
diagnosis of meckels diverticulum
Abdo USS
Xray for obstruction
diagnostic- meckel scan (99Tcm)
management of Meckels diverticulum
asymptomatic- nothing
- symptomatic- surgical excision
most common cause of cholestatic jaundice in first 3/12
biliary atresia
features of biliary atresia
prolonged cholestatic jaundice, acholic stools, dark urine and hepatomegaly.
Management of biliary atresia (pharm and non-pharm)
Pharm
- ursodeoxycholic acid (promotes bile flow)
- ADEK (fat soluble)
- nutritional support
- Abx- prophlyaxis for cholangitis
Surg- Kasai
- unless decompensated liver disease- then portoenterostomy or liver transplant if fails
what type of hypersensitivty reaction is IgE mediated CMPA
Type 1
Features of an IgE mediated allergic reaction
uriticaria/ angioedema/ lip/ tongue swelling
D&V/ nausea/ colicky abdo pain
Feautres of a non-IgE mediated food allergy
GORD
Blood/mucousy stool
colic
food aversion
perianal redness
constipation
what is Sandifer snydrome
abnormal posturing secondary to GORD
sudden deviation of head and neck to one side and the legs to the other
back typically arches
diagnosis of conjenital CMV infection
urine or saliva CMV polymerase chain reaction should be sent to confirm the diagnosis
treatment of wilsons disease
Treatment is with oral penicillamine as a copper-binding agent plus a low-copper diet (avoid excessive chocolate, shellfish and liver). Patients on penicillamine require vitamin B6 (pyridoxine) supplementation to avoid neuropathy
Presentation of Alagille syndrome
Alagille syndrome is a genetic disorder that can affect the liver, heart and bones. There is bile duct malformation with bile build-up in the liver, causing clinical jaundice and faltering growth. Congenital heart defects are also common and usually present with pulmonic stenosis, as well as bone abnormalities such as butterfly vertebrae.