4A: Abdominal Flashcards
(26 cards)
Leptospirosis/weils
get from animals, soil or water
acutely unwell
icteric
subconjunctival blood.
No stigmata of chronic liver disease,
List some inherited cystic kidney conditions.
PKD
Von-Hippel Lindau syndrome
Tuberous sclerosis
What system is used to classify renal cysts in polycystic kidney disease based on contrast-enhanced CT findings?
Bosniak System
Bosniak 1: Simple Cyst
Bosniak 4: Malignant
What is the prevalence of ADPKD?
1 in 1000
Accounts for 10% of renal replacement patients in the UK
How common are simple renal cysts?
2% of those aged 50 years
20% of elderly people
What should a cranial nerve palsy in the context of polycystic kidney disease make you suspicious of?
Intracranial aneurysm
What are the two mutations that can cause ADPKD?
PKD1 on Chromosome 16 (85%)
PKD2 on Chromosome 4
What are some differences between PKD1 and PKD2?
PKD1 is more severe and tend to lead to end stage kidney disease at a younger age. It is also MORE common.
How does polycystic kidney disease present?
Abdominal pain
Hypertension
Blood test abnormalities
Urinalysis abnormalities
Haematuria
UTI
Intracranial aneurysms
Genetic testing for family members
What are the main features of tuberous sclerosis?
Epilepsy
Learning disability
Autism
Hamartomas
Renal cysts
Angiomyolipomas (renal)
Shagreen patches
Ash-leaf spots
Adenoma sebaceum
Ash leaf spots
Adenoma Sebaceum
Shagreen patch(orenge peel)
In Tuberous sclerosis
How is ADPKD managed?
CONSERVATIVE
- Low salt diet
MEDICAL
- Antihypertensives (RAAS blockade)
- Tolvaptan limits cyst development
SURGICAL
- Remote problematic cysts and reduce mass effects
- Renal replacement therapy
What proportion of patients with ADPKD have extra-renal manifestations?
70% Liver Cysts
10% Pancreatic Cysts
5% Berry Aneurysms
Mitral valve prolapse
How does PBC tend to present?
Fatigue
Pruritus
Obstructive jaundice
What are the main features of Alport syndrome?
Deafness (bilateral and sensorineural)
Persistent microscopic haematuria
Proteinuria and CKD
Ocular abnormalities (e.g. lenticonus)
Leiomyomas
What are some clinical features of PBC?
Hepatosplenomegaly
Clubbing
Xanthelasma
Excoriation marks (from pruritus)
Jaundice
What are the main causes of chronic liver disease?
Alcoholic liver disease
Non-alcoholic fatty liver disease
Viral Hepatitis Hepatitis B and C
Autoimmune diseases: autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis.
Genetic diseases: hemochromatosis, Wilson’s disease, and alpha-1 antitrypsin
Drug-induced liver injury:
Vascular issues: Budd-Chiari syndrome,
Which antibody is associated with PBC?
Antimitochondrial (M2) antibody
Also associated with raised ALP and IgM
How is PBC managed?
MEDICAL
- Ursodeoxycholic Acid or Obeticholic Acid
- Cholestyramine for pruritus
SURGICAL
- Liver transplant (for intractable pruritus and end stage disease)
- Transplant has a good prognosis and low rate of recurrence
What are the main causes of chronic kidney disease?
Diabetes
Hypertension
Glomerulonephritis
Polycystic Kidney Disease
Chronic Pyelonephritis
Reflux Nephropathy
Which diseases are particularly at risk of recurrence in renal transplantation?
Focal segmental glomerulosclerosis
Amyloidosis
IgA Nephropathy
Haemolytic uraemic syndrome
What are some contraindications for renal transplantation?
Current malignancy
Severe poorly controlled comorbidity (e.g. COPD, heart failure)
Active infection (e.g. viral hepatitis)
Active substance misuse
Uncontrolled psychiatric disease
History of medication non-compliance
Features of decompensation of chronic liver disease?
Ascites,
jaundice,
hepatic encephalopathy
variceal bleeding
