Abdominal Flashcards
Extra renal manifestations of polycystic kidney disease
- Renal angle/loin pain
- Cyst infections
- Renal calculi
- Hypertension - excess renin release
- CKD
- Hepatic cysts
- Berry aneurysms
- Mitral valve prolapse
- Aortic regurgitation
- Diverticular disease
- Inguinal hernias
- Polycythaemia - excess Epo production
Inheritance of polycystic kidney disease
Autosomal dominent
Type 1: 80% - mutation Chr 16
- PKD 1 gene
Type 2: 20% mutation Chr 4
- PKD 2 gene
- less severe
- later onset
- fewer cysts
Indications for nephrectomy in PKD
- Recurrent infections
- Intractable pain
- Making space for transplant
Barriers to renal transplantation
- Unable to match
- Current or recent malignancy
- Ongoing deep seated infections
- Obesity
- Ongoing vasculitis
Causes of chronic liver disease
- Alcohol
- Infection
- Hep B, Hep C, CMV - Malignancy
- HCC
- mets - Auto-immune
- autoimmune hepatitis
- primary sclerosing cholangiitis
- primary biliary cirrhosis - Metabolic
- Haematomachrosis
- Wilsons disease
- Non alcoholic fatty liver disease (NASH) - Vascular
- Budd Chiari
- CCF - Alpha 1 anti-trypsin deficiency
Chronic liver disease Ix
- FBC/U&E/LFT
- Coag!
- Albumin
- USS liver
- AFP
- Primary biliary cirrhosis = anti-mitochondrial antibody (M2 antibody)
- Iron studies - ferritin, iron, TIBC (decrease)
- Wilsons = decreased serum copper, decreased serum caeruloplasmin
- Primary sclerosing cholangiitis = ANA/AMA/ANCA may be +ve
- Autoimmune hepatitis = ANA + anti-smooth muscle antibody
Functions of spleen
- Sequestration + phagocytosis of old and abnormal RBC
- Extra-medullary haematopoeisis
- Immunological
- Blood pooling
- 1/3 of platelets are held in spleen
Causes of splenomegaly
Infiltration:
- Malignant:
- myeloproliferative
- lymphproliferative
- lymphoma - Benign
- amyloidosis
- sarcoidosis
- thyrotoxicosis
- Gaucher’s disease (fat deposition disease)
Increased function
- Increased removal defective red blood cells
- spherocytosis
- thalassaemia
- early sickle cell
- nutritional anaemia - Hyperplasia secondary to infection
- malaria
- visceral leishmaniasis
- EBV (Glandular fever)
- subacute infective endocarditis
- TB
- schistomiasis
- - Disordered immunoregulation
- RA (Felty’s syndrome)
- SLE
- Amyloidosis
Defect in flow
- Portal hypertension
- Splenic vein thrombosis
Causes of massive splenomegaly
- Myelofibrosis
- CML
- Chronic malaria
- Leishmaliasis
- Gaucher’s disease
Genetic basis of Wilson’s disease
Mutation of ATP 7B gene
- key to transport of copper into secretory pathway of the cell for incorporation into copper-containing enzymes and excretion of excess copper in the bile
- mutation of gene leads to accumulation of free copper in hepatocytes
Hereditary haemorrhagic telangiectasia/Osler-Weber-Rendu syndrome
- presentations
- GI bleed
- Epistaxis
- Telangiectasia
- Complications of arteriovenous malformation
- lung
- brain
- liver
- bowel
- spine
Haemochromatosis clinical features
- Hepatomegaly/cirrhosis
- Triad of fatigue/arthralgia/gonadal dysfunction (pituitary infiltration)
- Type 1 diabetes (bronzed diabetes)
- Skin discolouration
- Cardiomyopathy
Haemochromatosis genetics
- Autosomal recessive
- Prevalence 1 in 200
- 1/10 people carry the gene
- HFE gene: most common allele C282Y
Contra-indications for liver transplant
- IVDU
- Current EtOH XS
- Previous/current malignancy
- Significant medical or psychiatric co-morbidities
5.
Varient syndromes for liver transplant
- Diuretic resistant ascites
- Intractable pruritus
- Recurrent cholangitis
4.
Kings College Hospital liver transplant criteria for acute liver failure
Arterial lactate >3.5 4 hours after resus OR pH <7.3 or lactate >3 after 12 hours
INR >6.5
Creatinine >300
Encephalopathy grade III or IV
Indications for splenectomy
- Trauma
- Hypersplenism
- ITP
- Haemolytic anaemia
- Vascular - aneurysm/clot of splenic artery
Post splenectomy complications
Acute = bleeding
Infection
- encapsulated bacteria: neisseria, strep pneumonia, haemophilus
- pre-op vaccination against the above: pneumococcal, Hib vaccine, Men C vaccine
- annual influenza vaccine
- life long penicillin
- anti-malarial if travelling abroad
Hereditary sphrerocytosis
- genetics
- cause of haemolysis
- clinical signs
- Ix
- Treatment
Genetics:
- autosomal dominent or recessive
- mutation in gene that codes for RBC membrane protein resulting in biconcave shape now being spherical
Cause of haemolysis
- extra-vascular haemolysis in spleen
Clinical signs
- triad: fatigue, anaemia, jaundice
- gallstones
Investigations
- Hb, MCV, bilirubin, LDH, direct coombs (negative - to check for autoimmune), haptoglobin (reduced)
- Blood film
- Osmotic fragility test
- EMA test - flow cytometry
Treatment
- folic acid
- splenectomy
- cholecystectomy