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PACES > Abdomen > Flashcards

Abdomen Flashcards

1
Q

How is haemochromatosis inherited?

A

Autosomal recessive

Inheritance of mutated HFE gene

(however there is variable penetrance - so presence of mutation doesn’t always lead to disease)

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2
Q

Investigations in haemochromatosis

A

Bedside:
- ECG: arrhythmia (from cardiomyopathy)
- Urine: glycosuria

Bloods:
- FBC (usually normal)
- LFTs, albumin, INR
- Ferritin and transferrin (both raised)
- HbA1c
- Genotyping

Imaging:
- US liver
- Fibroscan
- Ferriscan (specialised MRI)
- Echo to assess cardiomyopathy
- Plain films of affected joints

Liver biopsy not needed!

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3
Q

Management of haemochromatosis

A

MDT - Hepatology +/- haematology

Venesection
- Initiation to bring transferrin to an acceptable level
- Then maintenance venesection

Management of complications:
- T2DM
- Cardiomyopathy
- Cirrhosis (diuresis, HCC screening, nutrition etc)

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4
Q

Causes of haemochromatosis

A

Primary = hereditary haemochromatosis

Secondary = acquired haemochromatosis
- Seen in multiple transfusions e.g. haemoglobinopathy

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5
Q

Hereditary haemchromatosis: examination

A

Tanned skin

CBG monitor

CLD signs

May have hepatomegaly

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6
Q

Hereditary spherocytosis - presentation

A

Splenomegaly
Anaemia -> fatigue
Gallstone
Leg ulceration

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7
Q

Mechanism of haemolysis in hereditary spherocytosis

A

Abnormal protein coding resulting in spherical RBCs (instead of biconcave)

These cells are prone to haemolysis, especially in the spleen

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8
Q

Hereditary spherocytosis inheritance

A

autosomal dominant

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9
Q

splenomegaly DDx

A

Massive spleen:
- CML
- Myelofibrosis
- Malaria

Otherwise:
1) Lymphocytosis
- Lymphoma (acute or chronic)
- Reactive (infection eg EBV)

2) Extra-medullary haematopoiesis
- Chronic haemolytic anaemia eg hereditary spherocytosis

3) Inflammatory
- Felty’s syndrome (RA + splenomegaly + neutropenia)
- Amyloidosis (infiltrative)

4) Congestive
- Portal hypertension (usually with hepatomegaly)

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10
Q

Complications of hereditary spherocytosis

A
  • Gallstones
  • Chronic anaemia
  • Aplastic anaemia
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11
Q

Investigations in hereditary spherocytosis

A
  • FBC, U+Es, CRP, LFTs
  • Blood film
  • Split bilirubin

Haemolysis screen:
- increased reticulocytes
- raised LDH
- low haptoglobin
- normal DAT

Specific testing:
- EMA binding

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12
Q

Hereditary spherocytosis management

A
  • Folic acid supplementation
  • RBCs as needed

Severe cases:
- Splenectomy, typically with cholecystectomy
- Vaccinations again encapsulated organisms

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13
Q

Coeliac disease - investigations

A

Diagnostic:
- Anti-TTG antibodies with IgA levels
-can have false negative if IgA deficiency
-need to eat gluten for preceding 6 weeks
- If equivocal result - proceed to duodenal biopsy
- villous atrophy and crypt hypertrophy

General testing:
- FBC, U+Es, CRP, LFTs
- B12, folate, vitamin D

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14
Q

Coeliac disease: management

A

Patient education is key
Gluten eradication from diet (refer to dietitian)

Support with iron, B12 and vitamin D supplementation if needed

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15
Q

Complications of coeliac disease

A
  • Iron def anaemia
  • B12, folate, vitamin D deficiency
  • Functional hyposplenism (need winter vaccinations)
  • Malignancies: enteropathy-associated T-cell lymphoma and small bowel cancer
  • Skin: dermatitis herpetiformis
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16
Q

When is simultaneous pancreas-kidney transplant typically performed?

A

Usually T1DM

Where diabetes has led to end stage renal failure

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17
Q

Graft choice in SPK

A

Usually the pancreas and kidney are both donated from a single deceased donor (usually brainstem death)

Can also be pancreas transplant from a deceased donor + kidney transplant from a live donor

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18
Q

SPK - examination

A

Midline laparotomy scar AND Rutherford-morrison scar

There may be 2 Rutherford Morrison scars, usually pancreas in LIF but usually this wouldnt be palpable

19
Q

Serum ascites albumin gradient interpretation

A

SAAG >1.1g/L = portal hypertension
- Cirrhosis
- Congestive cardiac failure

SAAG <1.1g/L
- Nephrotic syndrome
- Malignancy
- Pancreatitis
- Infection e.g. TB

20
Q

Non invasive liver screen

A

Viral serology
- Hep B surface antigen
- Hep C antibody (IgG)
- Hep D
- Hep A and E - acute hepatitis

Autoimmune profile:
- ANA, AMA, anti-smooth muscle, anti-LKM
- Immunoglobulins

Metabolic screen:
- Ferritin and transferrin sats
- Raised copper and low caeruloplasmin
- Alpha 1 antitrypsin

21
Q

PSC investigations and management

A

Investigations:
- Typically normal antibodies
- Can be p-ANCA +ve
- Cholestatic LFTs with characteristic strictures on MRCP giving a ‘beaded’ appearance
- Autoimmune screen: coeliac, thyroid
- Imaging: US liver, MRCP

Management:
- UDCA
- ERCP and stenting of strictures
- Colonoscopy for malignancy screening
- HCC monitoringAN

22
Q

PBC investigations and management

A

Investigations:
- Cholestatic LFTs and AMA +ve
- US liver
- Consider MRCP
- Fibroscan
- Send thyroid, coeliac and sjogrens screen

Management
- UDCA
- If not responding, refer to tertiary centre for consideration of 2nd line agents e.g. OCA, BF

23
Q

Causes of palpable liver transplant

A

Size mismatch

Hepatitis - graft dysfunction (tender edge)

24
Q

Causes of splenomegaly

A

Massive: 5 M’s
- Myelofibrosis
- CML
- AML
- Malaria
- leishManiasis

Moderate:
- Myeloproliferative: AML, CML
- Lymphoproliferative: ALL, CLL
- Congestive (portal hypertension)
- Inflammatory e.g. amyloidosis

Mild:
- Haemolysis e.g. hereditary spherocytosis
- Infection e.g. EBV, bacterial endocarditis
- Inflammatory e.g. SLE

25
Q

Important negative in splenomegaly

A
  • Clinical anaemia
  • Clinical jaundice
  • Lympadenopathy
26
Q

Splenectomy indications

A

1) Trauma/splenic haemorrhage

2) Chronic haemolytic states e.g. thalassemia major, hereditary spherocytosis

3) ITP refractory to medical management

27
Q

Splenectomy complications

A

Peri-operative complications:
- Bleeding
- Infection
- Damage to surrounding structures

Post-operative:
- Thrombocytosis
- Overwhelming sepsis from encapsulated organisms

28
Q

How to prevent complications in splenectomy patients

A

Pre-operative:
- Vaccinations >2 weeks prior to surgery
- Prophylactic Abx

Post-operative:
- Patient education (attend hospital early if unwell)
- Medic alert bracelet
- Prophylactic Abx
- Vaccines e.g. pneumovax

29
Q

General management of CKD

A

1) Treat underlying cause:
- Control HTN, diabetes
- Remove nephrotoxic agents
- Treat glomerulonephritis where possible

2) Complications:
- Hypertension
- Hypervolaemia
- Electrolyte and acid-base disturbance
- Renal bone disease
- Renal anaemia
- Increased CVD risk

3) End-stage renal failure
- RRT: Haemodialysis, peritoneal dialysis, renal transplant
- 4-variable kidney risk factor gives 5-year probability of requiring RRT

4) Patient education
- Weight loss
- Smoking cessation
- Dietitians - low salt/potassium diet
- vaccinations

30
Q

Pharmacological management of CKD

A

Hypertension: ACEi

Hypervolaemia: diuretics (furosemide)

Electrolyte disturbance: potassium binder, phosphate binder

Renal bone disease: activated vitamin D, DEXA monitoring

Renal anaemia: iron replacement, EPO if affecting QOL

Cardiovascular risk: statin and anti-platelet

Also
- Stop nephrotoxics where possible
- Adjust meds to renal dosing

31
Q

Nephrectomy indications

A
  • Debulking surgery
  • RCC
  • Recurrent infections
  • Significant haematuria
32
Q

Management of PKD

A
  • Monitoring for CKD and treating complications
  • Tolvaptan (vasopressin receptor antagonist)
  • Consider RRT
  • Monitoring and assessment of complications e.g. saccular aneurysms, SAH
33
Q

Emergency indications for surgery in IBD

A

Toxic megacolon
Colonic perforation
Intra-abdominal abscess (if severely septic)

34
Q

Management of ascites

A

Fluid restriction
Low salt diet
Diuretics
Large volume paracentesis
TIPSS
Transplant

34
Q

Non invasive liver screen

A

LFTs incl. synthetic function (albumin, clotting)

Viral hepatitis serology + HIV

Autoimmune
- ANA, AMA (PBC)
- ANCA (PSC)
- SMA/LKM1 (AIH)
- Immunoglobulins
- A1AT

Metabolic:
- Ferritin and transferrin sats
- Copper, caeruloplasmin
- HbA1c and lipids

35
Q

Complications of TIPSS

A

Hepatic encephalopathy
Pulmonary HTN

36
Q

Bilateral renal masses DDx

A

Polycystic kidneys

Bilateral hydronephrosis

Bilateral renal massess e.g. angiomyolipoma in Tuberous Sclerosis

37
Q

Extra-hepatic complications of alcohol excess

A

Neuro:
- Cerebellar ataxia
- Alcohol-related brain damage
- Wernicke’s encephalopathy
- Korsakoff’s syndrome
- Peripheral neuropathy

Cardiac:
- Dilated cardiomyopathy

HPB/GI:
- Pancreatitis
- Malnutrition

38
Q

Prognostic scores in CLD

A

Child-Pugh score

UKELD

39
Q

Causes of gynaecomastia

A

Idiopathic

Drugs:
- Spironolactone
- Digoxin
- Anabolic steroids

Chronic liver disease

Hyperthyroidism

Geneic e.g. Klinefelters syndrome

40
Q

If you see a stoma in the lower left abdomen (not a colostomy)?

A

Could be a mucus fistula stoma
This is done to allow mucus to pass from disconnected bowel

41
Q

Risk stratification of needing RRT

A

4-variable kidney failure risk score

Gives probability of needing RRT in next 5 years

42
Q

Management of ulcerative colitis

A

ASUC:
- Bowel rest
- IV hydrocortisone

Maintenance:
- Mild-moerate: 5-ASA analogues e.g. mesalazine
- AZT
- Biologics e.g. infliximab
- Surgical resection

43
Q

Functions of the spleen

A

Lymphocytosis (honorary lymph node)
Extra-medullary haemopoiesis

PACES (5 decks)

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