Medical Abdomen Flashcards

1
Q

Chronic liver disease

On examination

Inspect: general, hands, face, trunk, ankles

Abdo: inspect/palpate

sig negatives!

A

Peripheral Inspection

  • General
    • Jaundice
    • Ascites
    • Cachexia
    • Tattoos and track marks
    • Pigmentation
  • Hands
    • Clubbing (esp. in PBC)
    • Leukonychia
    • Terry’s nails (white proximally, red distally)
    • Palmer erythema
    • Dupuytren’s contracture
  • Face
    • Pallor: ACD
    • Xanthelasma: PBC
    • Keiser-Fleischer rings
    • Parotid enlargement (esp. c¯ EtOH)
  • Trunk
    • Spider naevi
    • Gynaecomastia
    • Loss of 2O sexual hair
  • Ankles
    • Peripheral oedema

Abdomen

  • Inspection
    • Distension ± Para- / umbilical hernia
    • Dilated veins
    • Drain scars
  • Palpation
    • ± hepatomegaly
    • ± splenomegaly
    • Shifting dullness

Significant Negatives

  • Evidence of decompensation
    • Jaundice
    • Encephalopathy: asterixis, confusion
    • Foetor hepaticus: ammonia and ketones
    • Hypoalbuminaemia: oedema and ascites
    • Coagulopathy: bruising
  • Evidence of SBP: esp. if ascites
  • Cause: xanthelasma, pigmentation, KF rings, tattoos
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2
Q

Definition of Chronic liver disease

A

Progressive destruction of the liver parenchyma over a period greater than 6 months leading to fibrosis and cirrhosis

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3
Q

CLD

Differentials

(common/rare)

A
  • Common
    • EtOH
    • Viral
    • NASH
  • Rarer
    • Genetic: HH
    • AI: AH
    • Drugs: methotrexate
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4
Q

History Qs in CLD case

(cases)

A
  • EtOH
  • Sexual Hx, IVDU, transfusions
  • FH
  • Other AI disease: e.g. DM, thyroid
  • DH
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5
Q

Investigations in CLD case

Initial work up

(and 2 ‘other’)

A

Initial Workup

  • Urine: dip ± MC+S (? UTI)
  • Bloods: FBC, U+E, LFTs, INR, glucose
    • LIVER SCREEN!
  • Ascitic tap: chemistry, cytology, MC+S, SAAG
    • PMN >250mm3 indicates SBP
  • US + PV duplex
    • Liver size and texture
    • Focal lesions
    • Ascites
    • Portal vein flow
  • Other:
    • liver biopsy
    • MRCP: PSC
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6
Q

CLD

Liver screen

(7-think ‘cause’)

A
  1. EtOH: MCV, GGT, AST:ALT >2
  2. Viral: Hep B and C serology
  3. NASH: lipids
  4. AutoAbs: SMA, AMA, pANCA, ANA
  5. Ig: ↑IgG – AIH, ↑IgM – PBC
  6. Genetic: caeruloplasmin, Ferritin, α1-AT
  7. Ca: AFP, Ca 19-9
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7
Q

Treatment of CLD

General/specific (to cause)

A

General

  • MDT: GP, hepatologist, dietician, palliative care, family
  • EtOH abstinence
  • Good nutrition
  • Cholestyramine for pruritus
  • Screening: liver screen
  • HCC: US + AFP
  • Varices: OGD (propranolol+banding, acute UGI bleed: terlipressin)

Specific

  • HCV: protease (-) + ribavarin
  • PBC: ursodeoxycholic acid
  • Wilson’s: penicillamine
  • HH: venesection, desferrioxamine
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8
Q

Complications of CLD

(7)

A
  1. Varices: β-B, banding
  2. Ascites: fluid and salt restrict, spiro, fruse, tap, daily wt
  3. Coagulopathy: Vit K, FFP, platelets
  4. Encephalopathy: avoid sedatives, lactulose, rifaximin
  5. Sepsis / SBP: tazocin or cefotaxime
    • Avoid gent: nephrotoxicity
  6. Hypoglycaemia: dextrose
  7. Hepatorenal syndrome: IV albumin + terlipressin
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9
Q

Causes of CLD

(common-3/other-5)

A

Common

  1. Chronic EtOH
  2. Chronic HCV (and HBV)
  3. NAFLD / NASH

Other

  1. Congenital: HH, Wilson’s, α1ATD, CF
  2. AI: AH, PBC, PSC
  3. Drugs: Methotrexate, amiodarone, isoniazid
  4. Neoplasm: HCC, mets
  5. Vasc: Budd-Chiari, RHF, constrict. pericarditis
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10
Q

4 important complications of CLD

A
  1. Liver failure / decompensation
  2. SBP
  3. Portal HTN: SAVE (splenomegaly/ascites/varices/encephalopathy)
  4. HCC
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11
Q

Child-Pugh Grading of cirrhosis

A

Evaluates prognosis in Cirrhosis
Graded A-C using severity of 5 factors

  1. Albumin
  2. Bilirubin
  3. Clotting
  4. Distension: ascites
  5. Encephalopathy

A.5-6 = [0] 1 yr Mortality

B.7-9 = [20] 1 yr Mortality

C.10-15 = [50] 1 yr Mortality

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12
Q

CLD Decompensation precipitants

(HEPATICS)

A
  • Haemorrhage: e.g. varices
  • Electrolytes: ↓K, ↓Na
  • Poisons: diuretics, sedatives, anaesthetics
  • Alcohol
  • Tumour: HCC
  • Infection: SBP, pneumonia, UTI, HDV
  • Constipation (commonest cause)
  • Sugar (glucose) ↓: e.g. low calorie diet
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13
Q

General Management of CLD decompensation

(5)

A
  • HDU or ITU
  • Rx any precipitant
  • Good nutrition: e.g. via NGT c¯ high carbs
  • Thiamine supplements
  • Prophylactic PPIs vs. stress ulcers
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14
Q

Important monitoring in CLD decompensation

(3)

A
  • Fluids: urinary and central venous catheters
  • Bloods: daily FBC, U+E, LFT, INR
  • Glucose: 1-4hrly + 10% dextrose IV 1L/12h
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15
Q

Management of (6) Complications of decompensated CLD

A
  • Ascites: daily wt, fluid and Na restrict, diuretics, tap
  • Coagulopathy: Vit K, FFP, platelets
  • Encephalopathy: avoid sedatives, lactulose, rifaximin
  • Sepsis / SBP: tazocin or cefotaxime
  • Hypoglycaemia: dextrose
  • Hepatorenal syndrome: IV albumin + terlipressin
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16
Q

PAthophysiology of encephalopathy (CLD)

A

↓ hepatic metabolic function -> Diversion of toxins from liver directly into systemic system. -> Ammonia accumulates and pass to brain where
astrocytes clear it causing glutamate → glutamine -> ↑ glutamine → osmotic imbalance → cerebral oedema

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17
Q

Presentation of encephalopathy

(5)

A
  • Asterixis, Ataxia
  • Confusion
  • Dysarthria
  • Constructional apraxia
  • Seizures
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18
Q

Single key invesigation in encephalopathy (CLD)

A

Plasma NH4 (raised!)

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19
Q

Teatement of encephalopathy

A
  • Nurse in well lit, calm environment
  • Correct any precipitants
  • Avoid sedatives
  • Lactulose
    • ↓ nitrogen-forming bowel bacteria
    • 2-4 soft stools/d
  • Rifaximin PO: kill intestinal microflora
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20
Q

Hepatorenal syndrome

pathophysiology

(and classification)

A

Renal failure in pts. c¯ advanced CLF

Pathophysiology: “Underfill theory”
Cirrhosis → splanchnic arterial vasodilatation → effective
circulatory volume → RAS activation → renal arterial
vasoconstriction. Persistent underfilling of renal circulation → failure

  • *Type 1:** rapidly progressive deterioration (survival <2wks)
  • *Type 2**: steady deterioration (survival ~6mo)
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21
Q

Treatment of hepatorenal syndrome

A
  1. IV albumin + terlipressin
  2. Haemodialysis as supportive Rx
  3. Liver Tx is Rx of choice
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22
Q

SBP

Presenation

A

Pt. c¯ ascites and peritonitic abdomen
Complicated by hepatorenal syn. in 30%

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23
Q

SBP: Common organisms

A

Common organisms: E. coli, Klebsiella, Streps

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24
Q

SBP: 1 key investigation

A

ascites PMN >250mm3 + MC+S

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25
SBP management
taxocin or cefuroxime until sensitivities known prophylaxis: high recurrence therefore long term cipro given
26
Poor prognosis in CLD (4 factors)
**Worsening encephalopathy ↑ age ↓ albumin ↑ INR**
27
Ascites examination inspection: cause abdo completion sig negatives
* **Peripheral inspection: Cause** * Signs of **CLD** * **CCF**: ↑ JVP, bibasal creps, peripheral oedema * **Nephrotic**: periorbital oedema * **Budd Chiar**i: abdo pain, hepatomegaly, jaundice * **Abdomen** * Shifting dullness * Portal HTN: splenomegaly * **Completion** * CVS and Resp for CCF * Urine dip: proteinuria in nephrotic syndrome * **Significant Negatives** * CLD * Acute liver failure / decompensation * Cause: ↑ JVP, periorbital oedema
28
Causes of ascites 3: common 'SAAG'
**Commonest** 1. Cirrhosis 2. CCF 3. Carcinomatosis **Serum Ascites Albumin Gradient (**SAAG**)** = Se albumin – Ascites Albumin SAAG **_≥1.1g/dL_** = Portal HTN (97% accuracy) (transudate) * Cirrhosis in 80% SAAG **\<1.1g/dL** (exudate) * Neoplasia: e.g. peritoneal mets or ovarian Ca * Inflammation: pancreatitis * Nephrotic syndrome * Infection: TB peritonitis
29
Portal HTN Causes pre/hep/post
Portal pressure **_\>10mmHg_** (norm 5-10): 80% _cirrhosis_ in UK **Pre-hepatic** * Portal vein thrombosis * PV, ET (polycythemia vera (PV), essential thrombocythemia (ET)) * PNH * Nephrotic syndrome **Hepatic** * Cirrhosis **Post**-**hepatic** * Cardiac: RHF, TR, constrictive pericarditis * Budd-Chiari (hepatic vein thrombosis)
30
History Qs in ascites
* SBP: fever, abdo pain * Cause * Liver disease * Cardiac failure, MI
31
Key investigations in ascites Bedside/bloods/special/imaging
**Bedside** * Urine: dip ± MC+S * Exclude nephrotic syn. * ? UTI **Bloods** * FBC * U+E * LFTs: esp. albumin * INR * Glucose * Liver screen **Ascitic tap** * Chemistry * Cytology: malignancy, PMN (\>250/mm3 = SBP) * Bacteriology: MC+S, Ziehl-Neelson Stain * SAAG **US + PV duplex** * Liver size and texture * Focal lesions * Ascites * Portal vein flow
32
Treatment of ascites (general/diurectic/therapeutic paracentesis)
**General** * EtOH abstinence * Daily wts: aim for ≤0.5kg/d reduction * Fluid restrict: \<1.5L/d * Low Na diet: 40-100mmol/d **Diuretics** * Spironolactone * Add frusemide if response poor **Therapeutic Paracentesis** * Temporary insertion of pig-tail drain or Bonnano catheter * Indications * Respiratory compromise * Pain / discomfort * Renal impairment * Risks * Severe hypovolaemia: replenish albumin * SBP
33
Treatment of refractory ascites
TIPSS Transplant
34
Treatment/prophylaxis of SBP
* *Rx**: *Tazocin* or *cefotaxime* until sensitivities known * *Prophylaxis**: high recurrence -\> *cipro* long-term
35
Medical jaundice ## Footnote * examination (cause)* * Abdo* * completetion* * sig negatives*
**Cause** * CLD * Pancreatic Ca: cachexia, Virchow’s node * Haemolysis: pallor **Abdomen** * Excoriations and pruritus * Splenomegaly * Hepatomegaly * Palpable gallbladder: Ca head of pancreas **Completion** * Urine dip: look for BR, urobilinogen and Hb **Significant Negatives** * Acute liver failure / decompensation * CLD * Organomegaly
36
Differential for medical jaundic | (CLD/splenomeg/hepatomeg/none)
* **CLD**: EtOH, viral, NAFLD * **Splenomegaly** * Haemolysis * CLD ( → portal HTN) * Viral hepatitis: e.g. EBV * **Hepatomegaly** * Hepatitis * CLD * **No CLD or organomegaly** * Biliary obstruction * Haemolysis * Drugs: fluclox, OCP * Gilberts
37
Liver transplant Examination: peripheal general/immunosupp abdo sig negatives
**Peripheral Inspection** * **General** * Evidence of CLD * Pigmentation: HH * Tattoos and needle marks: Hep B/C * **Immunosuppressant Stigmata** * Cushingoid * Skin tumours: AKs, SCC (100x ↑ risk), BCC and MM * Gingival hypertrophy: ciclosporin **Abdomen** * Mercedes-Benz scar **Significant Negatives** * Evidence of CLD or cause for Tx * Evidence of immunosuppression * Absence of features of liver failure = working Tx **Differential for Mercedes Benz Scar** * _Hepatobiliary_ surgery * Liver _transplant_ * _Segmental resection_ * _Whipples’: pancreaticoduodenectomy_
38
History for liver transplant patient causes/Tx/current/DHx
* **Cause** * EtOH * Sexual Hx, IVDU, transfusions * FH * Other AI disease: e.g. DM, thyroid * DH * **Transplant** * adaveric or live segmental * Any complications * **Current health** * Acute rejection: fever, graft pain * Immunosuppression * Infection: e.g. CMV pneumonitis * Skin Ca * DH
39
Investigations for liver tranplant bloods/other
**Bloods** * FBC: infection * U+E: ciclosporin can → renal impairment * LFTs: assess graft function * Clotting * Fasting glucose: tacrolimus and steroids → DM * Drug levels: ciclosporin, tacrolimus **Other** * Liver biopsy: if rejection suspected
40
Top causes for liver tranplatnt (3)
* Cirrhosis * Acute liver failure * Hep A, B * Paracetamol overdose * Malignancy
41
Success of liver transplant
80% 1 year survival 70% 5 year survival
42
Liver transplant immunosuppression regime
Tacrolimus/ciclosporin azathioprine prednisolone (+/- withrawal @3mo)
43
Hepatomegaly * Examination:* * peripheral: causes* * abdo* * completion* * sig negatives*
* *Peripheral Inspection** * Cause* * CLD * EtOH: palmar erythema, Dupuytren’s * HH: skin discolouration * Ca: cachexia * CCF: ↑ JVP, bibasal creps, ascites, peripheral oedema * Haematological: pallor, bruising, purpura, LNs **Abdomen** * Hepatomegaly * Define fingerbreadths below costal margin at which liver edge palpable. * Moves inferiorly on inspiration * Can’t get above it * Dull PN * Features to note * Edge: smooth, craggy, nodular * Tenderness * Pulsatile * Percuss above and below to confirm enlargement * Auscultate for liver bruit * HCC * Other * Splenomegaly * Inguinal nodes * Ascites **Completion** * CVS and Resp: CCF **Significant Negatives** * Splenomegaly * Acute liver failure / decompensation * Cause: CLD, ↑JVP, pallor, bruising, LNs
44
Hepatomegalu common causes (3) Other causes (MACHO)
**Common Causes** * ***Hepatitis***: EtOH, viral, NAFLD * ***CLD*** * ***Congestion*** 2O to cardiac failure **Other Causes: MACHO** * ***Malignancy***: 2O * ***Anatomical***: Riedel’s lobe, hyperexpanded chest * ***Congestion***: TR, Budd Chiari * ***Haem***: leukaemia, lymphoma, myeloproliferative, SCD * ***Other***: sarcoidosis, amyloidosis, Gaucher’s, ADPKD
45
Key history Qs Hepatomegaly (3)
* **Hepatitis** / **CLD**: EtOH, viral exposure, FH * **Cardiac**: dyspnoea, PND, previous MI, rheumatic fever * **Haem**: tiredness, bruising, infections, bone pain
46
Hepatomegaly investigations bedide/bloods/imaging/histology
**Urine dip** * BR, urobilinogen * Proteinuria: amyloid **Bloods** * FBC * ↓Hb: malignancy, chronic disease * Lymphocytosis: hepatitis viruses, EBV (atypical) * U+E: CCF → renal impairment * LFT * Clotting * Liver screen **Imaging** * Abdo US + PV and hepatic duplex * Liver size and texture * Focal lesions * Ascites * Portal vein flow * Hepatic veins: thrombosis * CT: e.g. for tumour * MRI: good quality images of liver parenchyma **Liver Biopsy** * Check clotting first * Nodular Pattern * ***Micr***onodular: EtOH, HH, Wilson’s * ***Mac***ronodular: viral * Iron: Pearl’s stain * Copper: Rhodamine stain * α1ATD: PAS stain (α1AT globules accumulate in liver) * Amyloid: apple-green birefringence c¯ Congo Red * Granulomata: PBC
47
Causes of hepatomegaly | (6)
* **EtOH**: abstinence + support * **Viral**: supportive or anti-viral therapy * **HH**: venesection ± desferrioxamine * **Wilson’s**: penicillamine * **CCF**: ACEi, β-B and diuretics * **Haematological**: monitoring or chemo
48
Splenomegaly Examination Peripheral: cause abdo:inspect/palpate completion sig negatives
**Peripheral Inspection: Cause** * Haematological: pallor, bruising, purpura, LNs, cachexia * Cervical, axilla, inguinal * Portal HTN: CLD signs * IE: splinter’s, clubbing * Felty’s Syndrome: rheumatoid hands * *Abdomen** * Inspection*:Asymmetry * Palpation* * Splenomegaly * Can’t get above it * Moves inferiorly toward RIF on respiration * Notch * Dull PN * Not ballotable * Size: big or small? * Hepatomegaly? * Inguinal nodes? **Completion** * Cardio and Resp exam: IE and sarcoid * Urine dip: haematuria (IE), proteinuria (amyloid) **Significant Negatives** * Hepatomegaly * Haem: pallor, bruising, LNs * CLD * IE: splinters, clubbing * RA hands: Felty’s Syndrome
49
(isolated) Big spleen: limitted differential (4 categorieS)
* **Myeloproliferative**: CML, MF * **Lymphoproliferative**: CLL, lymphoma * **Infiltrative**: amyloidosis, Gaucher’s * **Developing world**: malaria, visceral leishmaniasis
50
Small spleen (common/other)
**Common** * Haem: myelo- / lympho-prolif disorders, haemolysis * Portal HTN: mostly 2O to cirrhosis * Infection: EBV **Other** * Infection: herpes viruses, hepatitis virus, IE, malaria * Inflammation: RA, SLE, Sjogren’s * Rare: sarcoidosis, amyloidosis, Gaucher’s, CVID
51
Hepatosplenomegaly:
same differentials as isolated splenomegaly (except inflammatory)
52
causes of MASSIVE splenomegaly
Causes of Massive Splenomegaly: \>20cm * C**M**L * **M**yelofibrosis * **M**alaria * Leish**M**aniasis * Gaucher’s (AR, glucocerebrosidase deficiency)
53
History Qs in splenomegaly (4)
* Haem: tiredness, bruising, infections, bone pain * CLD: EtOH, viral exposure, FH * Infections: fever, sore throat, jaundice, foreign travel * Inflammation: arthritis
54
Haematological Ix in splenomegaly splenomegaly= haematological + infective + liver Ix
* **FBC** * CML: ↑↑↑WBC – PMN, basophils, myelocytes * MF: pancytopenia * CLL: lymphocytosis * Haemolysis: ↓ Hb, ↑MCV, ↑RDW * **Film** * MF: leukoerythroblastic c¯ teardrop poikiolcytes * CLL: smear cells * Haemolysis: spherocytes, reticulocytosis * **Other Bloods** * DAT * U+E and urate: ↑ malignancy → uropathy * **Imaging** * Abdo US * CT chest and abdomen * PET scan * **Histology / Cytology** * LN biopsy * BM aspirate or trephine biopsy (MF) * **Genetic Analysis** * CML: Ph Chr, t(9:22) * MF: Jak2+ in 50%
55
Infective Ix in splenomegaly splenomegaly= haematological + infective + liver Ix
* **Urine Dip** * Haematuria: IE * **Bloods** * FBC: lymphocytosis (may be atypical in EBV) * U+E: renal impairment in IE * Thick and thin films * **Imaging** * Abdo US * Echo: IE
56
Liver Ix in splenomegaly splenomegaly= haematological + infective + liver Ix
* **Bloods** * FBC * LFT * Clotting * Liver screen * **Imaging** * Abdo US + PV duplex
57
Myloproliferative Disorders CML * symptoms* * genetics*
**CML** *Clonal proliferation of myeloid cells 15% of leukaemia* **Symptoms** * **Hypermetabolism**: wt. loss, fever, night sweats, lethargy * Massive **HSM** → abdo discomfort * Bruising / bleeding (platelet dysfunction) * Gout * Hyperviscosity ***Philadelphia Chromosome*** * *Reciprocal translocation: t(9;22)* * *Formation of BCR-ABL fusion gene* * *Constitutive tyrosine kinase activity* * *Present in \>80% of CML* * *Discovered by Nowell and Hungerford in 1960*
58
CML Investigations
* ↑↑WBC * PMN and basophils * Myelocytes * ± ↓Hb and ↓plat (accelerated or blast phase) * ↑urate * BM cytogenetic analysis: Ph+ve
59
CML management
* **Imatinib**: tyrosine kinase inhibitor * → \>90% haematological response * 80% 5ys * Allogeneic **SCT** * Indicated if blast crisis or TK-refractory
60
Primary myelofibrosis symptoms (/presentation)
*Clonal proliferation of megakaryocytes → ↑ PDGF →Myelofibrosis Extramedullary haematopoiesis: _liver and spleen_* **Symptoms** * Elderly * Massive **HSM** * Hypermetabolism: wt. loss, fever, night sweats * BM failure: anaemia, infections, bleeding
61
Investigations of myelofibrosis
* Film: leukoerythroblastic c¯ _teardrop_ poikilocytes * Cytopenias * BM: _dry_ tap (need trephine biopsy) * 50% _JAK2_+ve
62
Treatment of primary myelofibrosis
* Supportive: blood products * Splenectomy * Allogeneic BMT may be curative in younger pts.
63
Prognosis of myelofibrosis
5 yr median survival
64
Anatomy of the spleen
*Intraperitoneal structure lying in the LUQ Measures 1x3x5 inches Weighs ~7oz Lies anterior to ribs 9-11*
65
Function of spleen
**Function**: part of the mononuclear phagocytic system * Phagocytosis of old RBCs, WBCs * Phagocytosis of opsonised bugs: esp. encapsulates * Antibody production * Sequestration of formed blood elements * Platelets, lymphocytes and monocytes * Haematopoiesis
66
What is hypersplenism
* Pancytopenia due to pooling and destruction w/i an enlarged spleen. * Anaemia, bruising, infections * Sequestration crisis in SCD → hypovolaemic shock
67
causes of hyposplenism (4)
1. splenectomy 2. coeliac disease 3. IBD 4. SCD
68
Four findings on a bloodfilm indicative of hyposplenism
1. ↑ platelets transiently after splenectomy 2. Howell-Jolly bodies 3. Pappenheimer bodies 4. Target cells
69
Management of hyposplenism (3)
1. Immunisations: * Pneumovax * HiB * Men C * Yrly flu 2. Daily Abx: Pen V or erythromycin 3. Warning: Alert Card and/or Bracelet
70
Indications for splenectomy (6)
* Trauma * Rupture: e.g. 2ndary to EBV * AIHA * ITP * HS * Hypersplenism
71
Complications of splenectomy
* Redistributive thrombocytosis → early VTE * Temporary post-op aspirin prophylaxis * Gastric dilatation: transient ileus * May disturb gastro-omental vessel ligatures * Prophylactic NGT post-op * Left lower lobe atelectasis * Pancreatitis: tail shares blood supply c¯ spleen * ↑ susceptibility to infections * Encapsulates: haemophilus, pneumo, meningo
72
Enlarged kidneys Examination findings * peripheral: renal impariment/replacement/immsupp* * Abdo: inspect, palpate, ausc* * completion* * sig negatives*
**Peripheral Inspection** * Renal Impairment * HTN * Pallor * Renal Replacement Therapy * AV fistula (or scar) * Tunnelled dialysis lines (or scar) * Tenchkhoff catheter (or scar) * Immunosuppressant Stigmata * Cushingoid * Skin tumours: AKs, SCC, BCC and MM * Gingival hypertrophy: ciclosporin **Abdomen** * Inspection * Nephrectomy scar * Rutherford Morrison scar * Tenchkhoff catheter (or scar) * Palpation * Palpable Kidney * Flank mass * Can get above it * Ballotable * Moves inferiorly c¯ respiration * Resonant PN * Hepatomegaly * Renal Transplant * Auscultation * Renal bruit **Completion** * External genitalia: hydrocele 2O to RCC * Urine dip: proteinuria, haematuria * CVS: mitral valve prolapse **Significant Negatives** * Unilateral enlargement * Hepatomegaly * Evidence of RRT or immunosuppression
73
Enlarged kidneys differential | (bilateral, 4 + unilateral, 4)
**Bilateral** * ADPKD * Bilateral RCC (5%) * Bilateral cysts: e.g. in VHL * Amyloidosis **Unilateral** * Simple renal cyst * RCC * Compensatory hypertrophy * + contralateral nephrectomy: ADPKD
74
History Qs in enlarged kidneys (3 key)
* **ADPKD**: FH, loin pain, haematuria, headaches * **RCC**: haematuria, loin pain * **Compensatory hypertrophy**: previous infections, stones
75
Key investigations in enlarged kidneys bedside/bloods/imaging/other
**Urine** * Dip: haematuria, proteinuria * Cytology **Bloods** * FBC: ↓Hb 2ndary to ESRF * U+E: ↓eGFR, electrolyte disturbance * Bone profile: ↓Ca, ↑ PO4, ↑PTH **Imaging** * Abdo US * Confirm renal enlargement * Cysts * Hydronephrosis * Masses * Liver enlargement * CT/MRI * MRA: Berry aneurysms * CT abdo: esp. if RCC suspected **Other** * Genetic studies to look for mutation in * PKD1 gene on Chr 16: 85% * PKD2 gene on Chr 4: 15% * Family screen
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Management of ADPKD ## Footnote * general* * medical* * surgical*
**General** * ↑ water intake, ↓ Na, ↓ caffeine (may ↓ cyst formation) * Monitor U+E and BP * Genetic counselling * 50% chance of transmission * 10% are de novo mutations * MRA screen for Berry aneurysms **Medical** * Rx HTN aggressively: \<130/80 (ACEi best) * Rx infections **Surgical**: Nephrectomy * Recurrent bleeds or infections * Abdominal discomfort
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ADPKD progression...
*ESRF in **70**% by **70yrs** Mx complications: CRF HEALS _Dialysis_ or _transplant_*
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ADPKD Epidemiology
* Prev: 1:1000 * 5% of ESRF in UK * Genetics * PKD1 gene on Chr 16: 85% * PKD2 gene on Chr 4: 15% * Involve cell-cell interactions
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Presentation of ADPKD
* Age: 30-50s * HTN * Recurrent UTIs * Loin pain: cyst haemorrhage or infection * Haematuria
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Extra-renal involvement of ADPKD (3)
* Hepatic cysts → hepatomegaly * Intracranial Berry aneurysms → SAH * MV Prolapse: mid-systolic click + late systolic murmur
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ARPKD epidemiology prev/genetics
* *Prev**: 1:40000 * *Genetics**: PKHD1 (fibrocystin) gene on Chr6
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Presentation of ARPKD (4) | (and extra-renal involvement 1)
* Perinatal * Oligohydramnios: may → Potter sequence * Clubbed feet, pulm. hypoplasia, cranial abnormalities * Bilateral abdominal masses * HTN and CRF Extra-renal involvement: comgenital hepatic fibrosis-\> portal HTN
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Prognosis of ARPKS
ESRF by **20yrs!** NEED transplant
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Simple renal cyst
* Common: 1/3 of pts over 60yrs * May present as renal mass and haematuria * Contain fluid only: no solid elements * Main differential is RCC
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Dialysis associated with renal cysts
* Seen after prolonged dialysis * 2O to obstruction of renal tubules by oxalate crystals * ↑ risk of RCC in cyst: 15% of pts on haemodialysis
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Tuberous Sclerosis (Bourneville's disease) (3)
AD condition c¯ **_hamartomas_** in skin, brain, eye, kidney * *Skin**: nasolabial adenoma sebaceum, ash-leaf macules, peri-ungual fibromas * *Neuro**: ↓IQ, epilepsy, astrocytoma * *Renal**: cysts, angiomyolipomas
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RCC epidemiology (%/age/sex)
* *90**% of renal cancers * *Age**: 55yrs * *Sex**: M\>F=2:1
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RF for RCC (5)
* Smoking * Obesity * HTN * Dialysis: 15% of pts. develop RCC * 4% heritable: e.g. VHL syndrome
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Pathophysiology of RCC
``` *Adenocarcinoma from proximal renal tubular epithelium Clear Cell (glycogen) subtype: 70-80%* ```
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Presentation of RCC
50% incidental finding **Triad**: Haematuria, loin pain, loin mass Invasion of L renal vein → varicocele (1%) Cannonball mets → SOB
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Paraneoplastic features of RCC (5)
**EPO** → polycythaemia **PTHrP** → ↑ Ca **Renin** → HTN **ACTH** → Cushing’s syn. **Amyloidosis**
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Spread of RCC
* Direct: renal vein * Lymph * Haematogenous: bone, liver and lung
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Investigations of RCC | (blood/urine/imaging)
* **Blood**: polycythaemia, ESR, U+E, ALP, Ca * **Urine**: dip, cytology * **Imaging** * CXR: cannonball mets * US: mass * IVU: filling defect * CT/MRI
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Management of RCC Med/surg +prognosis
**Medical** * Reserved for pts. c¯ poor prognosis * _Temsirolimus_ (mTOR inhibitor) **Surgical** * Radical nephrectomy * Consider partial if small tumour or 1 kidney **Prognosis**: 45% 5ys
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Von Hippel-Lindau
* Autosomal Dominant * Renal and pancreatic cysts * _Bilateral_ renal cell carcinoma * Haemangioblastomas (Often in cerebellum → cerebellar signs) * Phaeochromocytoma * Islet cell tumours
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Renal tranplant Examination * Peripheral inspection: renal imp/replacement/immsupp/cause* * abdo:insp/palp/ausc* * completion* * sig negative*
**Peripheral Inspection** * Renal Impairment * HTN * Pallor * Renal Replacement Therapy * AV fistula (or scar) * Tunnelled dialysis scars * Tenchkhoff catheter scars * Immunosuppressant Stigmata * Cushingoid * Skin tumours: AKs, SCC (100x ↑ risk), BCC and MM * Gingival hypertrophy: ciclosporin * **Cause** * DM * Finger pricks from BM monitoring * Insulin marks on abdomen, lipodystrophy * Cystic Kidney Disease * Nephrectomy scars or ballotable kidneys * Connective Tissue Disease * SLE, SS, RA **Abdomen** * Inspect * Rutherford Morrison Scar in RIF * Nephrectomy scars * Tenchkoff catheter scars * Palpate * Smooth oval mass under scar * Dull PN * Can get below it * Doesn’t move with respiration * Auscultate * Renal bruit over transplant **Completion** * Dipstick: haematuria and proteinuria * Drug chart: any potentially nephrotoxic drug (e.g. ACEi) * BP: HTN common post-Tx **Significant Negatives** * Evidence of immunosuppression Signs of a cause: DM and PKD Working Tx Renal replacement therapy not in use No pain over Tx site
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Gum hypertrophy differential | (5)
* Drugs: *ciclosporin, phenytoin, nifedipine* * Familial * AML * Scurvy * Pregnancy
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History Qs in Renal Transplant
* **Cause** * DM * GN * **Transplant** * Cadaveric or live * any complications: e.g. urinary leaks, infection * **Current health** * Acute rejection: fever, graft pain * Graft function: Cr levels, BP, urine output * Immunosuppression * Infection: e.g. CMV pneumonitis * Skin Ca * CV risk * DHx
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Renal transplant Investigations *Urine/blood/other*
**Urine** * Dip: haematuria, proteinuria * MC+S **Bloods** * FBC: infection * U+E: eGFR – look @ trend * LFTs: ciclosporin can → hepatic dysfunction * Fasting glucose: tacrolimus is diabetogenic * Drug levels: ciclosporin, tacrolimus **Other** * Renal biopsy: if rejection suspected
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Commonest indications for renal transplant (4)
* Diabetic nephropathy * GN * Polycystic Kidney Disease * Hypertensive nephropathy
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Assessment of Renal Transplant | (pre-transplant)
* **Virology**: CMV, HIV, VZV, hepatitis * **Co-morbidities**: esp. CVD * **ABO** * **anti-HLA Abs**: may be acquired from blood transfusion * **Haplotype** * Importance: HLA-**DR** \> HLA-**B** \> HLA-**A** * 2 alleles @ each locus → 6 possible mismatches * ↓ mismatches → ↑ graft survival * Pre-implantation **cross-match** * Recipient serum vs. donor lymphocytes
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Contraindications to renal transplant
* Active infection * Cancer * Severe co-morbidity * Failed pre-implantation x-match
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Types of renal transplant grafts (4)
1. **Cadaveric**: brainstem death c¯ CV support 2. **Non-heart beating donor**: no active circulation 3. **Live-related** * Optimal surgical timing * HLA-matched * Improved graft survival 4. **Live unrelated**
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Immunosuppression in renal transplant | (pre/post-op)
* *Pre-op**: _campath_ / _alemtuzumab_ (anti-_CD52_) * *Post-op:** * Short-term: _prednisolone_ * Long-term: _tacrolimus_ or _ciclosporin_
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Prognosis for renal trasplant grafts Cadaveris vs HLA identical live
15yrs vs \>20yrs
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Complications ## Footnote *(post-op, 4/rejections, hyperacute/acute/chronic)*
**Post-op** * Bleeding * Graft thrombosis * Infection * Urinary leaks * *_Rejection_** * *Hyperacute** rejection: **minutes** * Path: ABO incompatibility * Presentation: thrombosis and SIRS **Acute** Rejection: **\<6mo** * Path: Cell-mediated response * Presentation * Fever and graft pain * ↓ urine output * ↑ Cr * Rx: Responsive to immunosuppression **Chronic** Rejection: **\>6mo** * Presentation: Gradual ↑ in Cr and proteinuria * Path: Interstitial fibrosis + tubular atrophy * Rx: supportive, not responsive to immunosuppression
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Drug toxicity in renal transplant ## Footnote *Ciclosporin/tacrolimus/steroids*
**Ciclosporin**: calcineurin inhibitor (blocks IL2 production) * Nephrotoxic: may contribute to chronic rejection * Gingival hypertrophy * Hypertrichosis * Hepatic dysfunction **Tacrolimus**: calcineurin inhibitor (blocks IL2 production) * \< nephrotoxicity cf. ciclosporin * Diabetogenic * Cardiomyopathy * Neurotoxicity: e.g. peripheral neuropathy **Steroids** → Cushing’s Syndrome and of course immunosuppression...
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SE of immunosuppression in renal transplant patients
↑ risk of **infection**: CMV, PCP, fungi, warts ↑ risk of **malignancy** * Skin: SCC, BCC, MM, Kaposi’s * Post-Tx lymphoproliferative disease (2O EBV)
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CVD in Renal patients
*Hypertension Atheromatous vascular disease A leading cause of death*
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Indications for renal replacement therapy (3)
* Suggested to start when GFR**\<15ml/min** + symptoms * Psychological preparation necessary * PD vs. HD depends on med, social and psych factors
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General dialysis complications
* 20% annual mortality * Cardiovascular disease * Malnutrition * Infection * uraemia → granulocyte dysfunction → ↑ sepsisrelated mortality * Amyloidosis * β2-microglobulin accumulation * Carpal tunnel, arthralgia * Renal cysts → RCC
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Haemodyalysis counter-current flow/ultrafiltration
**Counter-current flow** * Blood flows on one side of semipermeable membrane * Dialysate flows in the opposite direction on the other side. * Solute transfer by diffusion **Ultrafiltration:** *Fluid removal by creation of –ve transmembrane pressure by decreasing the hydrostatic pressure of the dialysate.*
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Complications of Haemodialysis
* Disequilibration syndrome (usually only 1st dialysis) * Rapid changes in plasma osmolarity → cerebral oedema * n/v, headache and ↓GCS * Fluid balance: BP↓, pulmonary oedema * Electrolyte imbalance * Aluminium toxicity (in dialysate) → dementia * Psychological factors
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Haemofiltration | (where is it used?)
*Usually only used in ITU Takes longer cf. HD but there is less haemodynamic instability.*
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Mechanism of haemofiltration
* Use a Vas Cath * Blood filtered across a highly permeable membrane by hydrostatic pressure and water and solutes are removed by convection. * Ultrafiltrate is replaced by isotonic replacement.
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Mechanism of peritoneal dialysis
* Dialysate introduced into peritoneal cavity by Tenchkhoff catheter. * Uraemic solutes diffuse into fluid across peritoneum * Ultrafiltration: addition of osmotic agent (e.g. glucose) * ~3L 4x /day c¯ ~4h dwell times
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Types of peritoneal dialysis (2)
Continuous Ambulatory Peritoneal Dialysis *CAPD: fluid exchange during **day** c¯ long dwell @ night* Assisted Peritoneal Dialysis​ *APD: fluid exchanged during **night** by machine c¯ long dwell throughout day.*
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Adbantages (3) and disadvantages (3) of peritoneal dialysis
**Advantages** * Simple to perform * Requires less equipment-\> *Easier @ home or on holiday* * Less haemodynamic instability -\> useful if cardio disease **Disadvantages** * Inconvenience * Body image * Anorexia
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Complications of peritoneal dialysis
* Peritonitis * Exit site infection * Catheter malfunction * Obesity (glucose in dialysate) * Mechanical: hernias and back pain
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AV fistula examination (inspection/palpation/austultation/sig negatives)
**Inspection** * Swelling c¯ surgical scar over distal forearm or @ elbow * Evidence of use: needle marks * Evidence of infection **Palpation** * Check if painful * Temperature * Palpable thrill **Auscultate** * Audible bruit **Significant Negatives** * Evidence of infection, stenosis, aneurysm
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Definition of AV fistula ## Footnote *(location,3/connection)*
***Surgically created connection between artery and vein*** * **Radio-cephalic** @ wrist = Cimino-Brescia * **Brachio-cephalic** @ the elbow Venous limb (from machine) is **proximal** Side-to-side anastomosis c¯ ligation of the distal vein
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Advantages (3) and Disadvantages (3) or AV fistula
**Advantages** * High flow rates, low recirculation (\<10%) * Low infection rates * Less chance of stenosis cf. grafts **Disadvantages** * Take ~6 weeks to arterialise * Affect pts. body image * Must take care: avoid shaving, don’t take BP/blood here
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Complications of AV fistula (4)
* Thrombosis and stenosis * Infection * Bleeding * Aneurysm
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Steal syndrome | (*AV fistulas*)
* Distal tissue ischaemia * Pallor, pain, ↓ pulses * May → necrosis * ↓ wrist:brachial pressure index * Rx: banding (plication)
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What is a tunnelled cuff catherer *(and AKA?)*
Two lines tunnelled under skin and entering **IJV** aka: Tessio Lines
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disadvantages of Tunnelled cuffed catheter for renal access
Disadvantages * May have ↑ recirculation cf. AVF * Lower flow rates * ↑ risk of infection and thrombosis *(advantages ==\>DON't have to...* 1. *wait ~6 weeks for AVF to arterialise* 2. *Suffer same affect on pts. body image* 3. *don't have to take care: e.g. avoid shaving, taking BP/blood from AVF arm)*
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Complication of Tunnelled cuffed Catheters (4)
* Adverse events @ insertion: e.g. pneumothorax * Line or tunnel infection * Blockage * Retraction
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Features and classification of CRF
* Kidney damage ≥3mo indicated by ↓ function * Symptoms usually only occur by stage 4 (GFR\<30) * ESRF is stage 5 or need for RRT
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Causes of CRF ## Footnote *Common, 2 Other, 7*
**Common**: * DM * HTN **Other** * RAS * GN * CTD: SLE, SS, RA * Polycystic disease * Drugs: e.g. analgesic nephropathy * Pyelonephritis: usually 2O to VUR * Myeloma and amyloidosis
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Investigations for CRF patient ## Footnote *urine/bloods: function+_renal screen_/imaging/histology*
**Urine** * Dip: haematuria, proteinuria, glycosuria * PCR * Normal = \<20mg/mM * Nephrotic = \>300mg/mM * BJP: myeloma **Function** * FBC: ↓Hb * U+E: ↓ eGFR * Bone: ↓Ca, ↑PO4, ↑PTH, ↑ALP **_Renal Screen_** * DM: fasting glucose, HbA1c * ESR * Immune * SLE: ANA, C3, C4 * Goodpasture’s: anti-GBM * Vasculitis: ANCA * Hepatitis: viral serology * Se protein electrophoresis **Imaging** * CXR: pulmonary oedema * Renal US * Usually small (\<9cm) * May be large: polycystic, amyloid * Bone X-rays: renal osteodystrophy (pseudofractures) * CT KUB: e.g. cortical scarring from pyelonephritis **Renal biopsy**: if cause unclear and size normal * Histology subtype * Amyloid: apple-green birefringence c¯ Congo Red
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Complications of CRF (*CRF HEALS)*
* *C**ardiovascular disease * *R**enal osteodystrophy * *F**luid (oedema) * *H**TN * *E**lectrolyte disturbances: K, H * *A**naemia * *L**eg restlessness * *S**ensory neuropathy
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Features of Renal osteodystrophy (5)
1. **Osteoporosis**: ↓ bone density 2. **Osteomalacia**: ↓ mineralisation of osteoid (matrix) 3. *2O/3O HPT* → ***osteitis fibrosa cystica*** * Subperiosteal bone resorption * Acral osteolysis: short stubby fingers * Pepperpot skull 4. **Osteosclerosis** of the spine → Rugger Jersey spine * Sclerotic vertebral end plate c¯ lucent centre 5. **Extraskeletal calcification**: e.g. band keratopathy
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Pathophysiology of renal osteodystrophy
* *↓ 1α-hydroxylase → ↓ vit D activation → ↓Ca → ↑PTH* * *Phosphate retention → ↓Ca and ↑PTH (directly)* * *↑PTH → activation of osteoclasts ± osteoblasts* * *Also acidosis → bone resorption*
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Management of CRF ## Footnote *General (and CV risk), Specific (HTN,Oedema,Bone,Anaemia,Restless)*
**_General Mx_** * Rx reversible causes * Stop nephrotoxic drugs * Na, K, fluid and PO4 restriction **Optimise CV Risk** * Smoking cessation, exercise * Statin + antiplatelet * Rx DM * *_Specific Mx_** * *Hypertension** * Target \<140/90 (\<130/80 if DM) * In DM kidney disease give **ACEi/ARB** (inc. if normal BP) * *Oedema**: frusemide * *Bone Disease** * Phosphate binders: calcichew, sevelamer * Vit D analogues: alfacalcidol (1 OH-Vit D3) * Ca supplements * Cinacalcet: Ca mimetic **Anaemia** * Exclude IDA and ACD * EPO to raise Hb to 11g/dL (higher = thrombosis risk) **Restless Legs**: clonazepam
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Diabetic nephropathy pathophysiology
*Commonest cause of ESRF: **\>20%** Advanced / ESRF occurs in **40%** of T1 and T2 DM* **Pathology** Diabetic nephropathy describes conglomerate of lesions occurring concurrently. * Hyperglycaemia → hypertrophy and ROS production * Hallmark is glomerulosclerosis and nephron loss * Nephron loss → RAS activation → HTN
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Clinical signs of diabetic nephropathy and screening test
* Microalbuminuria * 30-300mg/d or albumin:creatinine \>3mg/mM * Strong independent RF for CV disease * Progresses to proteinuria: albuminuria \>300mg/d * Usually coexists c¯ other micro- and macro-vasc disease * **Screening**: T2DMs should be screened for microalbuminuria 6moly
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Management of Diabetic nephropathy
* Good glycaemic control delays **onset** and **progression** * *UKPDS*: UK Prospective Diabetes Study * *DCCT*: Diabetes Control and Complications Trial * Control **HTN**: BP target **130/80** * **ACEi/ARB**: even if normotensive * Stop **smoking** * Combined kidney pancreas **Tx** possible in selected pts
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Rheumatoid arthritis association with CRF 3 processes
1. NSAIDs → ATN 2. Penicillamine and gold → membranous GN 3. AA amyloidosis occurs in 15%
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Pathogenesis of SLE association with CRF...and treatment
**Pathogenesis** * Involves glomerulus in 40-60% → ARF/CRF * Immune complex deposition → T3 hypersensitivity * Typically membranous GN * Proteinuria and ↑BP **Rx** * Proteinuria: **ACEi** * Aggressive GN: **immunosuppression**
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Diffuse systemic sclerosis and CRF association and treatment
* Renal crisis: malignant HTN + ARF * Commonest cause of death * Rx: **ACEi** if ↑BP or renal crisis
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Pathophysiology of myeloma and CRF
* *Excess production of monoclonal Ab ± light chains (excreted and detected in 60% as urinary BJP).* * *Light chains block tubules and have direct toxic effects → ATN.* * *Myeloma also assoc. c¯ ↑↑Ca2+*
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Presentation and treatment of CRF in myeloma
**Presentation** * ARF / CRF * Amyloidosis **Rx** * Ensure fluid intake of 3L/d to prevent further impairment * Dialysis may be required in ARF
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Cause of Renovascular disease
Cause * Atherosclerosis in 80% * Fibromuscular dysplasia * Thromboembolism * External mass compression
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Presentation investigation and management of Renovascular disease
**Presentation** * Refractory hypertension * Renal bruits * Worsening renal function after ACEi/ARB * Flash pulmonary oedema (no LV impairment on echo) * Other signs of PVD **Ix** * CT/MR angio * Renal angiography **Rx** * Rx medical CV risk factors * Angioplasty and stenting * **AVOID ACEi/ARB**
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IBD Examination ## Footnote * Peripheral: general/hands/eyes/mouth/legs* * abdo: inspection/palpation* * completion*
**Peripheral Inspection** * **General** * Often young female pt. * Laparotomy scars * Malnutrition or wt. loss * Cushingoid * Pallor * **Hands** * Clubbing * Leukonychia * Beau’s lines * **Eyes** * Pale conjunctivae * Iritis, episcleritis * Mouth * **​**Aphthous ulcers * Gingival hypertrophy (ciclosporin) * **Legs** * Erythema nodosum * Pyoderma gangrenosum **Abdominal** * **Inspection** * Scars * May be multiple and atypical in Crohn’s * Healed stoma sites * Healed drain sites * Stomas or healed stoma sites * Enterocutaneous fistulae * **Palpation** * Tendernes * RIF mass * ± hepatomegaly **Completion** * Inspect perineum for perianal disease * Examine for extra-intestinal features * Large joint monoarthritis * Sacroileitis * Bronchiectasis
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Differential diagnosis for IBD (4)
* Crohn’s * UC * Malabsorption: coeliac * Mid-line lap: FAP
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Important History Qs in IBD
* Symptoms * Wt. loss, fever, malaise * Abdominal pain * Diarrhoea, blood and/or mucus PR * Peri-anal disease: abscesses, fistulae * Extra-intestinal: EN, arthritis, iritis, gallstones, PSC * Therapy * Admissions * Medical therapy * Operations
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Investigations of IBS | (bloods, stool, imaging, endoscopy)
**Bloods** * FBC: ↓Hb, ↑WCC * U+E: dehydration, ↓K * LFTs: ↓ albumin, deranged LFTs * Clotting: ↑INR * ↑ ESR, ↑ CRP: used to monitor activity * Haematinics: Fe, B12, folate * ***_Markers of Activity in CD:_*** ↓Hb, ↑ESR, ↑CRP, ↑WCC, ↓albumin **Stool** * Culture + CDT: exclude infective causes * Campy, Yersinia, Shigella, C. diff, TB **Imaging** * AXR * Toxic megacolon in UC * Bowel obstruction 2O to strictures in Crohn’s * Contrast studies * Ba or Gastrograffin enema in UC * Small bowel follow-through in Crohn’s * MRI: perianal disease in Crohn’s **Endoscopy** * Ileocolonoscopy + regional biopsy * Ix of choice * Safe in acute disease * Distinguish UC from Crohn’s * Assess disease severity * Wireless capsule endoscopy
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Differentiate Pathology UC vs Crohns
153
Differential complications UC Vs Crohn's
154
Scoring system for severe exacerbation in IBD (3 key factors, 6)
Dx: **True-Love and Witts Criteria** 1. Symptoms * BMs \>6 x /d * Large PR bleed 2. Systemic Signs * ↑ HR \>90 * Pyrexia \>37.8 3. Laboratory Values * ↓ Hb \<10.5g/dL * ESR \>30mm/Hr
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Management of IBD Crohn's general/inducing remision/maintaining/surg (passmed)
**General points** * patients should be strongly advised to stop smoking * some studies suggest an increased risk of relapse secondary to NSAIDs and the combined oral contraceptive pill but the evidence is patchy **Inducing remission** * **_glucocorticoids_** (oral, topical or intravenous) are generally used to induce remission. * _enteral feeding with an elemental diet_ may be used in addition to or instead of other measures to induce remission, particularly if there is concern regarding the side-effects of steroids (for example in young children) * _5-ASA drugs_ (e.g. mesalazine) are used second-line to glucocorticoids but are not as effective * _azathioprine or mercaptopurine\*_ may be used as an add-on medication to induce remission but is not used as monotherapy. Methotrexate is an alternative to azathioprine * _infliximab_ is useful in **refractory** disease and fistulating Crohn's. Patients typically continue on azathioprine or methotrexate * _metronidazole_ is often used for **isolated peri-anal** disease **Maintaining remission** * as above, stopping smoking is a priority (remember: smoking makes Crohn's worse, but may help ulcerative colitis) * **_azathioprine or mercaptopurine_** is used first-line to maintain remission * _methotrexate_ is used second-line * _5-ASA_ drugs (e.g. mesalazine) should be considered if a patient has had previous surgery **Surgery**: around 80% of patients with Crohn's disease will eventually have surgery
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UC Management Inducing/maintaining remision (passmed)
**_Inducing remission_** * treatment depends on the extent and severity of disease * rectal (topical) **_aminosalicylates or steroids_**: for *distal colitis* **_rectal mesalazine_** has been shown to be superior to rectal steroids and oral aminosalicylates * oral **_aminosalicylates_** * oral prednisolone is usually used second-line for patients who fail to respond to aminosalicylates. NICE recommend waiting around 4 weeks before deciding if first-line treatment has failed * *severe colitis* should be treated in hospital. **_Intravenous steroids_** are usually given first-line **_Maintaining remission_** * oral **_aminosalicylates e.g. mesalazine_** * **_azathioprine and mercaptopurine_** * methotrexate is not recommended for the management of UC (in contrast to Crohn's disease) * there is some evidence that probiotics may prevent relapse in patients with mild to moderate disease
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IBD management surgery UC vs Crohns'
**Ulcerative colitis** * Elective indications for surgery include disease that is requiring maximal therapy, or prolonged courses of steroids. * Longstanding UC is associated with a risk of malignant transformation. Dysplastic transformation of the colonic epithelium with associated mass lesions is an absolute indication for a proctocolectomy. * Emergency presentations of poorly controlled colitis that fails to respond to medical therapy should usually be managed with a sub total colectomy. Excision of the rectum is a procedure with a higher morbidity and is not generally performed in the emergency setting. An end ileostomy is usually created and the rectum either stapled off and left in situ, or, if the bowel is very oedematous, may be brought to the surface as a mucous fistula. * Patients with IBD have a high incidence of DVT and appropriate thromboprophylaxis is mandatory. * Restorative options in UC include an ileoanal pouch. This procedure can only be performed whilst the rectum is in situ and cannot usually be undertaken as a delayed procedure following proctectomy. * Ileoanal pouch complications include, anastomotic dehiscence, pouchitis and poor physiological function with seepage and soiling. **Crohns disease** * Surgical resection of Crohns disease does not equate with cure, but may produce substantial symptomatic improvement. * Indications for surgery include complications such as fistulae, abscess formation and strictures. * Extensive small bowel resections may result in short bowel syndrome and localised stricturoplasty may allow preservation of intestinal length. * Staging of Crohns will usually involve colonoscopy and a small bowel study (e.g. MRI enteroclysis). * Complex perianal fistulae are best managed with long term draining seton sutures, complex attempts at fistula closure e.g. advancement flaps, may be complicated by non healing and fistula recurrence. * Severe perianal and / or rectal Crohns may require proctectomy. Ileoanal pouch reconstruction in Crohns carries a high risk of fistula formation and pouch failure and is not recommended. * Terminal ileal Crohns remains the commonest disease site and these patients may be treated with limited ileocaecal resections. * Terminal ileal Crohns may affect enterohepatic bile salt recycling and increase the risk of gallstones.
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Indications for surgery in IBD (5)
* Obstruction * Megacolon * Perforation * Severe GI bleeding * Failure to respond to medical therapy
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Key extra-intestinal features in IBD
* **Skin** * Clubbing * Erythema nodosum * PG (esp. UC) * **Mouth** Aphthous ulcers * **Eyes** * Anterior uveitis * Episcleritis * **Joints** * Large joint arthritis * Sacroileitis * Hepatic * Fatty liver * Chronic hepatitis → cirrhosis * Gallstones (esp. CD) * PSC + cholangiocarcinoma (esp. UC) * **Other** * AA amyloidosis * Oxalate renal stones
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Gen management UC vs Crohns gen/induction/maintenance