Gastro theory (gastro, renal) Flashcards
Peutz-Jeghers syndrome
-Autosomal dominant
-STK11 (LKB1) tumour suppressor gene
-dark freckles mucocutaneous (lips, mouth), oral mucosa and peripheries
-polyps GI c/o bleeding, intestinal obstruction, hronic pain
-high risk malignancy (breast, colorectal, pancreatic, stomach, ovarian, lung and small bowel)
DDx: hereditary hemorrhagic telangiectasia.
-epistaxis childhood
-red spots lips, tongue, fingertips
-FHx: Autosomal dominant. ENG, ACVRL1, MADH4 genes.
-AV malformations (pulmonary, cerebral)
Reversible causes of hepatic encephalopathy
Alcohol
Drugs
GI haemorrhage
Infection
Constipation
Indications for liver transplant
Acute
-paracetamol poisoning
-drug induced liver failure e.g. isoniazid, phenytoin, sodium valproate, MTX
-acute hepatitis
-EBV, CMV
Chronic
-alcoholic liver disease
-autoimmune hepatitis
-PBC
-PSC
-chronic viral hepatitis
-wilson’s disease
-budd-chiari syndrome
-hepatic malignancy
Stages of hepatic encephalopathy
1 sleep-wake cycle reversal
2 confusion
3 decreased level of consciousness, rousable
4 stupor, intubation
Amyloidosis
-extracellular deposition of abnormal proteins
-amyloid L (AL) and amyloid A (AA)
AL: abnormal light chain production ?myeloma
-cardiomyopathy, heart failure
-renal failure
-peripheral neuropathy
-malabsorption
-clotting function
-dx: bx, serum amyloid P (SAP) scan
-tx: myeloma and tx complications.
AA: abnormal deposition of serum amyloid A (SAA) protein, acute phase protein.
-secondary to inflammatory disorders (RA) and infection.
-liver
-spleen
-kdiney
-dx: bx
-mx underlying disorder
Crohns v Ulcerative colitis
Crohns:
-distribution: patchy “skip” lesions
-depth: transmural
-area: whole GI tract, terminal ileum and anus
-smoking: higher risk
-fistulae and stenosis: common
-Mx: conventional, then infliximab and adalimumab for severe active dx.
UC:
-distribution: continuous
-depth: superficial
-area: large bowel, rectum
-smoking: lower risk
-fistulae and stenosis: rare
-Mx: steroids, mercaptoprine or AZA. Mod-severe: infliximab and golimumab.
Consider vedolizumab for both.
Extra-intestinal manifestations of IBD
-Skin: erythema nodosum, pyoderma gangrenosum, aphthous ulceration
-Joints: seronegative arthritides (large-joint arthritis, sacroiliitis)
-Eye: uveitis, episcleritis, scleritis, conjunctivitis
-Hepatobiliary: PSC (more likely in UC), cholangiocarcinoma
-Renal: oxalate stones
Screening prior to biologics
Hx and exam (TB features)
Hepatitis serology
HIV test
CXR
T-spot test (quantiferon test if pt not on steroids)
Renal stones
-calcium oxalate
-hydroxyapatite
-uric acid
-struvite
-cystine
calcium oxalate: underlying metabolic disorder
Hydroxyapatite: no metabolic abnormality
Uric acid: idiopathic hyperuricaemia or hyperuricosuria
Struvite: renal infection
Cystine: renal tubular defect
Von Hippel-Lindau syndrome
Autosomal dominant
VHL gene (tumour suppressor gene)
Haemangioma
Angiomata retina (retinal haemorrhage, visual loss), brain, spinal cord, liver, kidney, pancreas
Cerebellar haemangioblastoma (lateral lobes)
Phaeochromocytoma
RCC
Endolymphatic sac tumours (inner ear, hearing loss, tinnitus, balance problems)
Annual screen: eye, kidney, urinary peptides for phaeo, intermittent brain imaging.
Mx: surgical removal, cryoablation or laser, external beam radiotherapy.
Renal transplant notes.
Investigation
-bloods: renal function, FBC, bone scan, LFT, CRP (infection), blood culture, virology (BK virus and CMV PCR), immunosuppression levels (toxicity)
-urine: dipstick (haematuria, proteinuria, leucocytes, nitrates), urine mcs, urine quantification for proteinruia, virology for BK virus
-US (doppler vessels) ?obstruction, renal perfusion, renal artery stenosis, renal vein thrombosis
-transplant biopsy: r/o rejection ?other cause
Signs of transplant failure
1. declining renal function
2. proteinuria
3. tenderness over graft
4. fluid overload
5. interstitial fibrosis, tubular atrophy or vascular changes on biopsy
Management: calcineurin inhibitor + antiproliferative agent + steroids
CNI:
-tacrolimus: tremor, alopecia, diabetes, HTN, nephrotoxicity, renal failure, blood disorders
-ciclosporin: gingival hypertrophy, hirsutism / hypertrichosis, hyperlipidemia (AND as for tacrolimus).
Antiproliferative:
-Mycophenolate mofetil: N/V/D, leucopenia, lymphopenia, anaemia, thrombocytopenia, foetal toxicity, infection, malignancy (skin), PTLD
-Azathioprine: hypersensitivity reactions, dose related bone marrow suppression, liver impairment, infection, pancreatitis
-Prednisolone: thin skin, easy bruising, muscle wasting/weakness, diabetes, HTN, osteoporosis, pancreatitis, infections, glaucoma, cataracts, heart failure
-Rapamycin inhibitors (mTOR): sirolimus, evelorimus. HTN, impaired healing, VTE, ILD, proteinuria, hyperlipidemia, reversible male infertility.
Alport syndrome
-genetics
-manifestations
-management
Genetics:
Type IV collagen defect
X-linked (M > F), AD and AR
Manifestations:
Kidneys: microscopic haematuria, proteinuria
Ears: sensorineural hearing loss
Eyes: anterior lenticonus (progressive visual loss)
Management:
-ACE inhibitors or ARBs (delay onset ESKD)
-Hearing aids
PBC v PSC
Primary biliary cirrhosis
Primary sclerosing cholangitis
PBC = small bile ducts
. autoimmune, AMA (anti-mitochrondrial ab)
. urso
PSC = med - large bile ducts
. assoc UC
. ANA/ASMA
. urso not that great
. endoscopic stricture, bile acid sequestrants.
CKD staging (D)
- eGFR notes
- eGFR >90
- eGFR reduction >60
- eGFR >30
- eGFR >15
- eGFR <15 (kidney failure)
eGFR notes:
- Average measure of stable creatinine over days, not for AKI.
- Elderly w/out proteinuria + eGFR >45 fine. eGFR decreases with age.
- Black x 1.2.
- Not accurate in pregnancy.
- Over-estimates if low muscle mass (or limb amputation)
IDEAL study, eGFR 7-10 to commence dialysis. AV fistula for HD need 3/12 post-op to use (eGFR 15-20).
CKD presentation + complications.
Presentation-
- nocturia
- lethargy
- loss of appetite
- fluid retention
- pruritis
Complications-
Pericarditis, serositis, encephalopathy, GI bleeding, uraemic nephropathy.
CKD Risk factors
- causes
- risk for progression
- trigger precipitating renal failure
Causes-
- DM- bx if no micro/mascopic complications of DM.
. complications.
. ACE / ARB.
. creatinine increase < 30%, need for renoprotection / increase >30%, stop drug.
- GN.
- Interstitial nephritis.
- HTN. Dx, duration and control of HTN, tx and compliance with medications, angiography and FHx. Angioplasty for artheromatous renal artery stenosis / sympathetic denervation = medical tx.
- PKD.
- Reflux nephropathy. Childhood renal infections, cystoscopy, operations, tx (abx), enuresis.
- Analgesia nephropathy
- Uncertain
Risk for progression: low birth weight, HTN, AKI, proteinuria, smoking, hyperuricaemia, increased glomerular pressure (pregnancy, obesity, diabetes)
Trigger-
- meds (NSAIDs, trimethoprim, aminoglycoside)
- radiocontrast
- infection
- ACE / ARB (if bilateral renal art stenosis)
- dehydration
- anaemia
CKD mx.
Fluid intake
Diet- protein / potassium restriction
Anaemia- EPO.
Acidosis
Phosphate / calcium / bones
Cardiovascular risk reduction
- HTN mx.
1. ACEI/ ARB. Expect creat rise.
2. Loop diuretic for fluid.
3. Dihydropyridine CCB > thiazide.
4. Aldosterone antagonists, monitor serum potassium.
5. Loop + thiazide, reduce potassium.
- Smoking cessation and avoiding nephrotoxins.
Consider vascular access
Dialysis timing
Transplant compared to conservative mx
Specific.
- ANCA associated vasculitis. Rituximab (induction) -> azathioprine (maintenance).
- SLE immunosuppression.
- thrombotic microangiopathy. ADAMTS13 TTP and eculizumab for HUS.
- PKD tolvaptan.
- Bladder mx for reflux or neurogenic bladder.
- Social arrangements and ADLs: employment, family coping, financial, travel, sexual function.
Glomerulonephritis.
Presentation: proteinuria, haematuria, oliguria, oedema, sore throat, sepsis, rash, haemoptysis. Kidney bx. E.g. IgA nephropathy for ACE / ARB then steroids if acute.
PRIMARY
A. Diffuse
1. minimal change (children)
2. membranonoproliferative (Hep C, autoimmune- SLE, scleroderma, infections like malaria or syphilis, essential cryoglobulinaemia, malignancies, drugs like penicillamine, NSAIDs, anti-TNF, mercury or gold poisoning).
3. proliferative (post-strep, mesnagiocapillary, crescenteric, mesangioproliferative).
B. Focal
1. IgA nephropathy (HIV, chronic liver dx, IBD, coeliac). Haematuria, consider post mild-URTI. Ddx: bladder tcc, kidney stones.
2. Focal segmental glomerulosclerosis (FSGS, nephrotic syndrome). Causes- primary, familial, HIV, morbid obesity, heroin, reflux nephropathy.
SECONDARY (SLE, GPA, PAN, Goodpasture’s syndrome, Henoch-Schonlein purpura, infective endocarditis, cryoglobulinaemia + Hep C, myeloma, DM, haemolytic uraemic syndrome).
Acute interstitial nephritis.
Allergies.
- PPI, penicillin, NSAIDs, gadolinium contrast.
Immune.
- transplant rejection, autoimmune nephritis.
Infections.
- TB, bacterial pyelonephritis, leptospirosis.
Toxins.
- mushrooms, myeloma light chains.
Nephrotic v Nephritic syndrome.
Nephrotic- glomeruli protein leak.
- proteinuria (>3.5g/d)
- hypoalbuminaemia (<30)
- oedema
- hyperlipidemia (increased LDL and cholesterol)
-thrombosis
Causes-
primary (membranous GN >40yo, FSGS, MPGN, minimal change)
secondary
-systemic dx- DM, SLE,
-hodgkin’s- minimal change
-solid tumor-membranous, amyloid, multiple myeloma
-infection- hep B-membranous, HIV- IgA nephropathy, collapsing focal sclerosis, infective endocarditis
-drugs- d-penicillamine, probenecid, NSAIDs, heroin
Ix: urine protein, lipids, casts.
Blood: creatinine, albumin, lipid profile.
Secondary causes: HBA1c, ANA, hepB/C, protein electrophoresis, cryoglobulins, RF, syphilis (rapid plasma regain), HIV, complement.
US
Renal bx
Phospholipids A Recpetor
Nephritis- glomeruli inflammation and injury (protein, red and white blood cells leak into tubule).
- decreased filtration / renal function
- proteinuria
- haematuria, pyuria
- HTN
Causes- IgA nephropathy, SLE (low C3/4), pauci-immune crescenteric GN, MPGN (low C3 or C4), thrombotic microangiopathy (low C3), post-infectious GN (low C3), anti-GBM goodpasture’s, C3 glomerulonephropathy (low C3).
ADPKD
FHx (AD).
PC: haematuria, polyuria, loin pain, HTN, headache and visual disturbance (intracranial aneurysm).
Extra-renal manifestations:
- liver cysts / hepatomegaly
- pancreatic cysts
- splenic cysts
- thyroid cysts
- seminal vesicle cysts
- intracranial cerebral aneurysm
- hypertension
- diverticular disease
- hernias.
Ddx- alport syndrome (hearing loss, kidney loss, eye loss).
CKD complications.
Anaemia
Bone disease
Secondary gout or pseudogout
Pericarditis
HTN
Cardiac failure
Fluid overload
Peripheral neuropathy
Pruritis
Peptic ulcers
Impaired cognition
Poor nutrition
Renal transplant contraindications.
Absolute.
- cancer <2 years since remission
- severe IHD
- active infection
- vasculitis / anti-GBM dx
- occlusive aortoiliac dx
Relative.
- >75yo
- other co-morbidities
- high risk dx recurrence in transplant
- ureteric or bladder dx (ileal conduit pre-transplant)
Renal transplant rejection.
Very early (hrs-days)
- renal a. or v. thrombosis (surgical problems, thrombophilia or SLE)
- ureteric leak (small bladder)
- delayed graft function (long graft ischemia time, older donor, elevated tacrolimus level)
- hyperacute rejection (HLA-mismatch, previous transplant, pre-formed anti-HLA ab)
Early (weeks) (HLA mismatch, previous transplant or pre-formed HLA ab)
- acute rejection
- non-adherence to treatment or inadequate immunosuppression
Months (ureteric stent, intense immunosuppression, donor kidney dx, graft damage while harvesting)
- renal a. stenosis
- BK virus nephropathy
Years (insufficient immunosuppression, non-adherence, previous acute rejections)
- chronic allograft injury (ab-mediated, tx with plasmapheresis, immunoglobulin infusions or different immunosuppressives such as rituximab)
Any time.
- cyclosporin or tacrolimus toxicity (high doses, serum levels not monitored, CYP450 inhibiting drugs)
- infection
- original kidney dx recurrence (minimal change- early, IgA nephropathy or membranous- later, FSGS).
- diabetics w. additional pancreatic tplant (pancreatic drainage, bladder or to gut and leak).
EPO and iron level
Hb<100 (aim 100-115)
Tsat > 20%
Ferritin > 200
Contraindications: malignancy (adenocarcinoma)
Risk: stroke, HTN, thrombosis
CKD MBD
Vit D
Calcium
PTH
-secondary or tertiary hyperparathyroidism
Low phos, potassium diet
Cinacalcet or parathyroidectomy
Dialysis
-Social / quality of life
-Physical
-Prognosis
Sleeping
Carpal tunnel syndrome
Fertility and sexual dysfunction
Education
Employment
Travel and logistics
Mood and cognition
QoL
Support
Cardiovascular risk factors
Metabolic profile
Vaccination
Transplant
Prognosis, end of life care planning
