Abdominal Flashcards
Genetic causes of PCKD
Mostly AD
PCKD1 - Chromosome 16 80%
PCKD2 - Chromosome 4 20% - tends to be less severe and later progression
Presentation of ADPCKD
Hypertension
Renal failure or worsening renal function
Proteinuria/haematuria
Extra renal manifestations
Extra renal manifestations of ADPCKD
Hypertension
Cysts in liver, pancreas or seminal vesicles
Cererbal aneurysms - haemorrhage
Colonic diverticuli
Management of PCKD
Management of HTN - ACEi
Hyperlipidaemia mx
High fluid low salt diet
Vasopressin antogonists eg tolvaptan may be used
RRT/dialysis
Indications for nephrectomy in PCKD
Make room for transplanted kidney
Progression to RCC
Chronic pain/infection
Large and significant haematuria
Clinical signs in Chronic Liver disease
Hepatomegaly
Spider naevi
Bruising
Corneal arcus
Loss of axillary hair
Palmar erythema
Dupytren’s contracture
Caput medusae and splenomegaly (signs of portal hypertension)
Asterixis, ascites, and jaundice - decompensation
Related to causes
- Tattoos - viral hepatitis
- Diabetes - NAFLD
- Xanthelasma - PBC
Causes of CLD
Alcoholic liver disease
Non-alcoholic fatty liver disease
Viral hepatitis
autoimmune disorders autoimmune hepatitis, PSC, PBC
Haemachromatoiss
Wilsons disease
HHT
A1AT
Drugs
Investigations in new CLD
History
FBC, U&Es, LFTs, albumin, Coag
GGT, AST
BBV
Ferritin and caeruloplasmin
Autoantibody screen - ANA, AMA, ASMA, LKM
tumour markers - AFP
CXR - if concern re CCF
USS
CT abdo/pelvis
Fibroscan
Ascitic tap if ascites
Potential endoscopy - varies, portal hypertension gastropathy
Biopsy - transjugular if ascites present
Antibodies in PBC
Elevated AMA and IgM
Antibodies in Autoimmune hepatitis
IgG, ASMA and Anti-LMK1
Symptoms in PBC
Tiredness and fatigue
Pruritis
Liver failure
Complications of PBC
CLD and cirrhosis
Malignancy - HCC
Treatment in PBC
UDCA
Liver transplant
Antibodies in PSC
pANCA
Common causes of ESRF
Diabetes
Hypertension
PCKD
Glomerulonephritis
When to approach renal transplantation
Approaching ESRF but not requiring dialysis
- better prognosis if not on dialysis
Use kidney failure risk equation
ESRF definition
eGFR <15ml/min
Barriers/contraindications to kidney transplan
Not available matching donor
Malignancy
Deep seated infection
Uncontrolled vasculitis
Obesity
Side effects of long term immunosuppression
Infection/malignancy particularly SCCs and PTLD
Steroids - skin thinning, easy bruising, cushingnoid appearance, infection, GI bleeds, osteoporosis, diabetes
Cyclosporin - hirsutism, gingival hyperplasia
Tacrolimus - Tremor
Causes of hepatomegaly
Alcoholic liver disease
NAFLD
Viral Hepatitis
Haemochromotosis
Malignancy
Congestive cardiac failure
Ix of hepatomegaly
Bloods inc glucose and INR
Iron studies
Liver screen
HIV and BBV
USS + marking if ascites present
Ascitic tap
Fibroscan
SAAG
> 1.1g/L - systemic process eg cardiac failure, portal hypertension (cirrhosis and Budd chair), nephrotic syndrome, Meig’s syndrome
<1.1g/L - Malignancy/pancreatitis/TB
Ascitic fluid analysis
Albumin
Protein
Glucose
Cell count and gram stain
Amylase - pancreatitis
Causes of CLD
Cirrhosis (alcoholic)
Carcinoma
Congestion - CCF and Budd-chiari
Infection - Viral hepatitis
Immune - PBC, PSC, AI
Infiltrative - Amyloid, Haemoinfiltrative disorder
Iron - Haemochromotosis
Medications - methotrexate
Liver function indicative of alcoholic hepatitis
AST:ALT ration >2
Identification of chronic pancreatitis - hx of abdominal pain in alcoholic liver disease
Faecal elastase
Mg - Low
Serum albumin - low indicates poor prognosis
Vitamin D - Low
Why is peritoneal dialysis avoided in ADPCKD
Large volume of fluid required in PD with large kidneys
Increased risk of cyst infection
Causes of splenomegaly
Portal hypertension
Haemotological disorders
- Lymphoma and lymphatic leukaemia
Myeloproliferative diapers, polycythaemia rubra ver and myelofibrosis
- Haemolytic anaemia and congenital spherocytosis, sickle cell
Infections
- Glandular fever
- Malaria
- Leishmaniasis
- Brucellosis
- TB
- Subacute endocarditis
Rheumatological:
- Feltys syndrome
- SLE
Sarcoidosis
Amyloidosis
Thyrotoxicosis
Gauchers disease
Ix of splenomegaly
Bloods
Autoimmune screen
HIV screen
USS abdo
Thick and thin films
Blood films
DAT
CTTAP - ?lymphoma
Bone marrow biopsy and trephine
Causes of massive splenomegaly
Myelofibrosis
Myeloid leukaemia
Malaria
Kal Azar
Causes of hepatosplenomegaly
Lymphoma
Myeloproliferative diseases
Cirrhosis with portal hypertension
Amyloidosis
Sarcoidosis
Glycogen storage disease
Tests in hereditary spherocytosis
FBC - anaemia
Blood film - spherocytes and haemolysis
Increased reticulocytes
Haemolysis screen - increased LDH and split bilirubin and reduced haptoglobin
LFTs
Coombs test
Osmotic fragility test
Flow cytometry - EMA binding
Complications of immunosuppression
Infection
Skin malignancy
Seborrhoic warts/actinic keratoses
Hypertension
Nephrotoxicity
Indications for liver transplant
Haemochromatosis
Drug induced liver injury - paracetamol
Alcoholic liver disease - in abstinent patients
NAFLD
Autoimmune - AI, PBC or PSC
Chronic viral hepatitis
HCC
Wilsons disease
A1AT
Variant syndromes - diuretic resistant ascites, chronic hepatoencephalopaty, intractable pruritus, hepatopulmonary syndrome, recurrent cholangitis
Suitability for liver transplant
Significant liver dysfunction
MDT approach
UK model for end stage liver disease (UKELD)
Ix of haemochromatosis
Routine blood
Ferritin
Transferrin saturations
Genetic testing - mutations in HFE gene
Immunosuppression side effects
Steroids
- Insulin injection sites/glucose testing
- Striae
- Cushinghoid face
Tacrolimus
- Tremor
Ciclosporin
- Gum hypertrophy
Contraindications to liver transplant
IV drug abuse
Alcohol excess - abstinence mandatory for ALD
Significant medical or psychiatric issues
History of prior malignancy
Age not a contraindication but poorer outcomes >65
UKELD score
> 49 - suitable for transplant
NA
Creatinine
INR
Bilirubin
Indications for liver transplant following paracetamol overdose
pH 7.25 24hrs post OD with adequate fluid resuscitation
OR
Creat>300
PT >100s
Grae 3/4 encephalopathy
Complications of liver transplant
Acute or chronic rejection
Biliary leaks and strictures
Immunosuppression side effects
Metabolic syndrome
Avoid live vaccines
Recurrence of hepatic disease e.g. PBC, viral hepatitis or Budd-Chiari syndrome
Causes of ascites
Vascular:
- Portal hypetension
- Budd chiari
- CCF
- Restrictive pericarditis
Low albumin
- Nephropathy
- Protein losing enteropathy
Peritoneal disease
- Meigs syndrome
- Infectious pertonitis - TB or fungal disease
- Malignancy - ovarian/gastro-intestinal
Miscellaneous
- Pancreatic leak
- Chylous ascites
- Peritoneal dialysis related ascites
- Advanced hypothyroidism
Inheritance of hereditary haemochromatosis
Autosomal recessive - usually due to mutations within HFE gene
Presentation of hereditary haemochromotosis
Screening
Raised ferritin
Arthralgia, sexual dysfunction and lethargy
Diabetes
Cardiomyopathy
Slate grey pigmentation of skin
Hepatomegaly
Polcythaemia
Screening in hereditary haemochromotosis
Screening in 1st degree relatives
Baseline ferritin - >200 in females, >300 in males
Transferrin saturation >40% in females and >50% in males
HFE defect - note have variable penetrance
Complications of hereditary haemochromotosis
Diabetes
Liver cirrhosis - will need USS 6/12 and AFP
Dilated cardiomyopathy
CPPD - pseudo gout
Treatment of hereditary haemochromotosis
Weekly venesection until ferritin level acceptable
Thereafter regular venesection
Management of diabetes, liver cirrhosis cardiomyopathy
Abstinence from alcohol
HCC surveillance
Joint findings in haemochromotosis
Typically affects MCPs in hands
Will have squaring of the joints
Hook like osteophytes
Chondrocalcinosis on XR
Ix in hereditary haemochromotosis
FBC - polycythaemia
LFTs - usually normal
INR
Albumin
Urine dip - glycosuria
ECHO
X rays of joints
USS liver +/- AFP
HFE gene analysis
Liver biopsy not needed for diagnosis but can be used to assess degree of fibrosis
Inheritance of hereditary Spherocytosis
Autosomal dominant in 5 genes that code for protein in the red blood cell membrane
Presentation of hereditary spherocytosis
Anaemia
Jaundice
Splenomegaly
Gallstones
Screening of 1st degree relatives
Neonatal jaundice
Complications of hereditary spherocytosis
Aplastic crises
Anaemia
Gallstones
Treatment of hereditary spherocytosis
Folic acid
Complications of anaemia - BTFs
Splenectomy - will need vaccine prior esp meningococcal, Hib and pneumococcal, medic alert bracelet
Prophylactic antibiotics
Cholecystectomy
Mechanism of haemolysis in hereditary spherocytosis
RBCs spherical shape instead of concave shape - undergo haemolysis in spleen
Splenectomy reduces haemolysis
Indications for splenectomy
Trauma - rupture
Haematological - ITP and hereditary spherocytosis
Investigations in coeliac disease
FBC - infection and anaemia
U&Es and LFTs
Anti-TTG while on gluten diet
TFTs
OGD with Jejunal biopsy - villous atrophy
Management in coeliac disease
Dietician
Education to avoid gluten products
Types of kidney transplant
Live (related or altruistic) or deceased (post circulatory or neurological death)
Drainage of transplanted pancreas
Historically into bladder to measure lipase as function of graft - risk of reflux pancreatitis and UTI
Now more usually drains in small bowel
Success in combined cadaveric pancreatic and kidney transplant vs live single kidney transplant
Similar survival at ten years, better survival beyond that in combined procedure
Long term effects in diabetes in combined transplant
Nephropathy - does not progress
Neuropathy - does not progress
Retinopathy - unclear
Complications in combined renal-pancreas transplant
Acute and chronic rejection
Graft thrombus
Graft pancreatitis
Infection
Islet cell transplantation vs pancreas transplant
Can be done in T1DM without nephropathy or chronic pancreatitis requiring total pancreatectomy
Islets infused into the portal vein - cadaveric used in T1DM, own islets used in pancreatitis
Islet cell transplant less invasive and less morbidity
Higher rate of insulin independence in pancreas transplant
Prognostic scores in cirrhosis
Child’s Pugh score - presence of ascites and encephalopathy, INR, bilirubin and albumin
MELD score - dialysis, INR, creatinine, sodium, bilirubin and INR - estimated 3 month mortality
Treatment for ascites
Fluid restriction
Diuretics e.g spironolactone
Ascitic drainage with albumin cover
TIPSS
Liver transplant
Complication in TIPSS
Issues with coagulopathy in patients
5-10% of patients will become encephalopathic following procedure
Causes of gynaecomastia in males
Anabolic steroid abuse
Medications such as spironolactone or eplerenone or digoxin
Testicular atrophy or
Klinefelters
Chronic liver disease
Complications of cirrhosis
Varices and ahemorrhage
Ascites with infection
HCC
Hepatorenal syndrome
Hepatopulmonary syndrome - vasodilation in the lungs of patients with CLD
Complications in chronic alcohol use
Parotid swelling - fatty infiltration
Cardiac problems - dilated cardiomyopathy
Hypertension
Pancreatitis
Gastric ulceration
UGI cancers
Cerebellar atrophy
Polyneuropathy
Wernicke’s encephalopathy
Korsakoff’s syndrome
Osteoporosis
What is wernicke’s encephalopathy?
Exhaustion of thiamine (B1)
Triad of ophthalmoplegia (most commonly affecting the lateral rectus muscle), ataxia and confusion
Added to Korsakoffs where there is confabulation
Causes of unilateral kidney enlargement
PCKD (other kidney not palpable or contralateral nephrectomy)
Renal Cell carcinoma
Simple cysts
Hydronephrosis
Causes of bilateral kidney enlargement
PCKD
Bilateral RCCs (in 5%)
Bilateral hydronephorsis - retroperitoneal fibrosis or bladder tumours
Amyloidosis
Tuberous sclerosis - renal angiomyolipomata and cysts
Von hippel Lindau
Causes of pancreatitis
Gallstones
Alcohol
Trauma
Medications - steroids and azathioprine
ERCP
Hypertriglyeridaemia
Hypercalcaemia
Cystic fibrosis
PRSS1 and SPINK1 - hereditary
Complications of pancreatitis
Necrotising pancreatitis
Sepsis
ARDS
Death
Chronic pancreatitis
Pseudocyst formation
Portal vein thrombosis
Pancreatic diabetes (type 3)
Pancreatic ductal stricture
Malabsorption
Duodenal or biliary obstruction
Increased risk of pancreatic cancer
Indicators of pancreatic exocrine insufficiency
Weight loss
Steatorrhea
Vitamin D deficiency
Hypomagnesaemia
Low faecal elastase
What is splenomegaly
Ix in IBD
Bloods:
- FBC
- U&ES and LFTs
- CRP
- Blood cultures
- Folate, B12
- Coeliac serology
Stool cultures
Faecal calprotectin
AXR
Colonoscopy/endoscopy via stoma
Differences between Crohns and UC
UC:
- Distally from rectum, affecting the colon only
- Not full thickness, only affects mucosa
- Associated with PSC
Crohns:
- Can affect anywhere along the GI tract
- Granulomatous disease
- More associated with fistula and abscess formation
- Full thickness transmural disease
Indications for emergency surgery in IBD
Toxic megacolon
Haemorrhage
Perforation
Tx in UC
Mild to moderate disease:
- 5-aminosacilylates - oral and rectal
- Oral steroids if no response
Moderate to severe
Acute severe:
- Iv fluids
- High dose steroids - IV Hydrocort
- pLMWH
- IA CRP >45 or the stool frequency >8 at day 3 are bad prognostic signs and senior review and/or surgical review should be undertaken immediately.
- Daily abdominal film whilst on IV steroid therapy and arrange surgical review if transverse or ascending colon diameter >6cm
Maintaining remission:
- 5-AS (mesalazine)
- oral or topical steroids (budesonide)
- If two or more flares in a year requiring remissions may need escalated to azathioprine or inflixamab or adalimumab
Criteria for severe flare in IBD
> 6 bloody stools per day and systemic toxicity with at least one of:
- temperature >37.8°C
- pulse >90bpm
- haemoglobin <105g/L or C-reactive protein >30mg/L
Indication for liver transplant
Chronic:
- ALD - if abstinent >6 months
- NASH
- Haemochromatosis
- Autoimmune hepatitis
- PBC
- PSC
- Chronic viral hepatitis
- Wilson’s disease
- A1AT disease
- HCC
Emergency
- Paracetamol overdose
- Fulminant viral hepatitis
Signs of decompensated liver disease
Evidence of jaundice - scleral icterus
Hepatic encephalopathy - confusion and asterixis
Ascites - shifting dullness
Evidence of varices - caput medusae and rectal varices on PR exam
Splenomegaly
Define acute liver failure
Acute impairment of liver function with encephalopathy occurs within 8 weeks of onset of symptoms, with no recognised chronic liver disease
Variant syndromes for listing for liver transplant
Diuretic resistant ascites
Chronic hepato-encephalopathy
Intractable pruritis
Hepato-pulmonary syndrome
Polycystic liver disease
Recurrent cholangitis
Contraindications to liver transplantation
IV drug abuse
Alcohol abuse - will need abstinence if ALD
Poor PS
Significant medical or psychiatric co-morbidities
Survival in >65 year much reduced
Poor malignancy within last 5 years
Indications for liver transplant in paracetamol overdose
pH <7.25 24 hrs post overdose after fluid resuscitation
PT >100s
Creat >300 or anuric
Grade III-IV encephalopathy
Complications of liver transplant
Episodes of rejection
Complications from immunosuppressive medication
- Increase risk of infection
- Increased risk of malignancy - skin and PTLD
- Metabolic syndrome - diabetes, obesity
- Avoid live vaccines
Biliary leaks and strictures
Recurrence of primary liver disease
- PBC and budd-chiari syndrome
Contraindications to renal transplant
Mismatched donor kidney
Recent or active malignancy
Deep seated infection
Active vasculitis
Declining in a capacities patient
Complications of chronic NSAID use
Increase CVS risk - MI and stroke
Gastritis
UGI ulcers and GI bleeds
CKD and AKI
Causes of membranous nephropathy glomerulonephritis
Idiopathic - usually phospholipase A2 ab is positive
SLE
Infections - malaria, Hep b, Hep C
Drugs - captopril, NSAIDS, penicillamine, anti-TNF therapy
Gold
Cancer
Causes of FSGS
Idiopathic
Genetic - Alport and Fabry’s
HIV
Steroid abuse
Lithium
Causes of minimal change
Idiopathic
Tumours
NSAIDS
Causes of RPGN
GPA
Goodpasture’s
SLE
Clinical signs in renal transplant
Arteriovenous fistula(e)
- Thrill (palpable or auscultated) - currently working
- Thrill and cannulation points/dressings - being used
- Failed
Neck - tunnelled dialysis line or previous scars
Abdominal scars:
- Iliac fossa scars - kidney transplant
- Flank scar - nephrectomy
- Peritoneal dialysis scars
Fluid status
Top reasons for renal transplant
Diabetes
ADPCKD
Glomerulonephritis
Things to note in a renal transplant presentation
Underlying cause for renal disease:
- Polycystic kidney disease - ADPCKD
- Visual impairment, skin prick marks, injection sites/pump - diabetes
- Sclerodactyly - systemic sclerosis
- Rheumatoid hands - rheumatoid arthritis
- (Hepato)splenomegaly - amyloidosis
- Ungal fibromata, adenoma sebaceous, polycystic kidneys - tuberous sclerosis
Current treatment modality
- Haemodialysis - working AV fistula with evidence of recent access, tunnelled lines
- Peritoneal catheters
Functioning transplant
Complications of past/current treatment
- Side effects of treatment of the underlying disease e.g. Cushingoid appearance (glomerulonephritis)
- Side effects of immunosuppressive in transplant patients
– Fine tremor - tacrolimus
– Gum hypertrophy - ciclosporin
– Hypertension - CNIs
– Steroid side effects
– Skin damage and malignancy - cyclosporin and azathioprine
Scar in kidney-pancreas transplant
Lower midline abdominal incision with palpable kidney in iliac fossa but no overlying scar
Problems following kidney transplantation
Rejection - acute or chronic
Infection secondary to immunosuppression:
- PJP
- CMV
Increased risk of other pathology
- Skin malignancy
- PTLD
- Hypertension and hyperlipidaemia causing cardiovascular disease
Recurrence of original disease - seen most in FSGS and IgA nephropathy
Chronic graft dysfunction
Success of renal transplantation
1 year graft survival - 95%
5 year graft survival - 50% (better with living-related donor grafts)
Success of liver transplantation
90% 1 year survival
80% 5 year survival
Causes of hepatosplenomegaly
Congestion - CCF
Cirrhosis
Carcinomatosis
Infection - hepatitides
Immune - AIH, PSC, PBC
Infiltrative - amyloid and myeloproliferative disorders
Evidence of hepatic decompensation on exam
Asterixis
Encephalopathy
Ascites
Complications of cirrhosis
SBP
Hepatic encephalopathy
Varices secondary to portal hypertension
Elements of a liver screen
Coag and albumin
Autoantibodies - ANA, Anti-SMA, Anti-LMK, Anti-Mitochondrial, ANCA
Hepatitis B and C
Ferritin and haematocrit
Caeruloplasmin
A1AT
AFP
Classification score in liver cirrhosis
Child Pugh Score - prognostic based on bilirubin/albumin/IMR/ascites/encephalopathy
A - 1 year survival 100%
B - 1 year survival 81%
C - 1 year survival 45%
Autoantbodies in liver disease
AIH - Anti-SMA and anti-LKM and ANA
PSC - ANA and cANCA
PBC - Anti-mitochondrial and IgM
Complications of haemochromotosis
Cirrhosis
- May necessitate liver transplantation
Restrictive cardiomyopathy
- May lead to CCF
Testicular atrophy and hypogonadism
Endocrine disturbance:
- Bronze diabetes
Pseudogout
Treatment in haemochromatosis
Venesection - initially until iron deficient then increased frequency
Avoid alcohol
Surveillance for HCC
Normal life expectancy without cirrhosis and effective treatment
Indication for splenectomy
Rupture - trauma
ITP
Hereditary Spherocytosis
Ix in hereditary spherocytosis
FBC - anaemia
Blood full
Unconjugated bilirubinaemia
EMA test - flow cytometry
Management in splenectomy patients
Vaccinations - to encapsulated organisms
- Ideally 2 weeks prior to surgery
- Pneumococcus
- Meningococcus
- Hib
Life long prophylactic penicillin
Medic alert bracelet
Issues following renal cell transplantation
Rejection:
- Acute or chronic
Infection (secondary to immunsuppression)
- PCP
- CMV
Secondary malignancy:
- Skin malignancy
- PTLD
Issues arising from underlying disease:
- Hypertension and hyperlipidaemia
Recurrence of original disease:
- Seen most commonly in FSGS and IgA nephropathy
Chronic graft dysfunction
Risk equation for needing transplant in kidney failure
The kidney failure risk equation
Uses gender, age, eGFR and UACR to calculate 2 and 5 year risk of progression to kidney failure requiring dialysis or transplant
Scores fo characterising chronic/acute liver failure
Chronic:
- UKELD - suitability for transplant - >49 mortality figures support transplant
- MELD - Mortality in three months
- Childs-Pugh - prognostic score
Acute:
- Kings college criteria
