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6 Gastroenterology
Non- alcoholic fatty liver disease (
NAFLD
CNS
LFT
OHCS
MRI
https://t.me/med1917
The most comm on liver disorde r in Wester n countrie s (preval ence ≈30
the leading cause of chronic liver disease worldwide,
the hepatic manifestation of the metabolic syndrome. It will soon overtake
the most common indication for liver transplantation (
hepatocytes (steatosis) visualized, eg on ultrasound that cannot be attributed to
other causes (most commonly alcohol so consider
The patient Most patients are asymptomatic, but some report persistent fatigue,
right upper quadrant pain or malaise, and hepatomegaly is common on exam. If
inflammation is also present (
NASH)
. Rule out other causes of liver disease (p
(
bolic disorders (obesity, dyslipidaemia, diabetes, hypertension). Progression to cirrhosis may occur— biopsy or elastography may be needed (
Risk factors for progression age; obesity; DM;
Tre at me nt Control risk facto rs includi ng obes ity; weight los s is the mai nstay of man-
agement (bariatric surgery helps). Address cardiovascular risk (commonest cause of
p85). Avoid alcohol consumption. No drug is of proven benefit or licences in
death, see
UK
, though vitamin E may improve histology in fibrosis (eg
the
associated with excess mortality). Pioglitazone improves fibrosis and inflammation
in patients with concurrent
NASH
in observational studies. Foll ow- up Monitor for complications (
DM
). If cirrhotic, screen for
Wilson’s disease/ hepatolenticular degeneration
Wilson’s disease is a rare (3/
deposition in liver and
Genetics An autosomal recessive disorder of a copper transporting
Physiology Total body copper content is ~
proximal small intestine). In the liver, copper is incorporated into caeruloplasmin. In
Wilson’s disease, copper incorporation into caeruloplasmin in hepatocytes and excretion into bile are impaired. Copper accumulates in liver, and later in other organs.
Signs Children present with liver disease (hepatitis, cirrhosis, fulminant liver failure);
young adults often start with
dystonias; Parkinsonism; ataxia/ clumsiness.
libido; personality change.
the doctor who is good at combining the analytical and integrative aspects will be
the first to make the diagnosis.
sions; mutism.
Haemolysis; blue lunulae (nails); arthritis; hypermobile joints; grey skin.
Tests Equivocal copper studies need expert interpretation.
1
Urine: 24h copper excretion is high, eg >
2
: non- specific (but
3
Serum copper: typically <11µmol/ L.
4
Serum caeruloplasmin: <
dental low values in protein- deficiency states (eg nephrotic syndrome, malabsorption).
5
Molecular genetic testing can confirm the diagnosis.
6
Slit lamp exam: KF rings: in iris/ Descemet’s membrane (see fig
7
Liver biopsy: hepatic copper (copper >
8
: degeneration in basal ganglia, frontotemporal, cerebellar, and brainstem.
Management Diet Avoid foods with high copper content (eg liver, chocolate,
nuts, mushrooms, legumes, and shellfish). Check water sources (eg wells, pipes) for
Drugs Lifelong penicillamine (
copper.
SE
: nausea, rash,
urinary copper and protein excretion.
Screen siblings Asymp tomati c homoz ygotes need treat ing.
disease.
Prognosis Pre- cirrhotic liver disease is reversible;
no clear clinical prognostic indicators. Fatal events: liver failure, bleeding, infection.
CNS
Kayser– Fleischer (KF) rings Copper in iris; they are not invariable. Also
WCC
, Hb, platelets, haematuria, nephrosis, lupus. Monitor
)
%) and now
NAFLD
LFT
, typically
15 can be thought of as
p
273
). It represents fat in
NAFLD
if drink <
ALT
) = non- alcoholic steatohepatitis
280
) and check for associated meta-
p
244
NASH
).
.
18U/ wk, <
HCV
as
9U).
800IU/ d— higher doses
DM.
Daily aspirin appears to reduce risk of progression to
HCC
wit h ultrasou nd ±
100 000
) inherited disorder of copper excretion with excess
AFP
twice- yearly.
NASH,
cirrhosis,
(eg basal ganglia). It is treatable, so screen all with cirrhosis.
ATP
ase,
ATP 7B
125
mg. Intake ≈ 3mg/ day (absorbed in
signs: tremor; dysarthria, dysphagia; dyskinesias;
.
Mood Depression/ mania; labile emotions;
Ignoring these may cause years of needless misery: often
Cognition Memory; slow to solve problems; IQ; delu-
100
ALT
>
1500
is not part of the picture).
200
mg/ L (<
500
Liver transplantation (See p
mcg/ 24h (normal <40mcg).
140
mg/ L is pathognomonic)— beware inci-
4.53
p
250
mcg/ g dry weight); hepatitis; cirrhosis.
368
mg/ 6– 8h PO for 1yr, maintenance 0.75– 1g/ d).
FBC
273
CNS
damage less so. There are
and
.) If severe liver
281
).
6 Gastroenterology
Liver tumours
https://t.me/med1917
282
The most common (90%) liver tumours are metastases (fig
bronchus, or the gastrointestinal tract (
much less common and may be benign or malignant (
Symptoms Fever, malaise, anorexia, weight,
Jaundice is late, except with cholangiocarcinoma. Benign tumours are often asymptomatic. Tumours may rupture causing intraperitoneal haemorrhage.
Signs Hepatomegaly (smooth, or hard and irregular, eg metastases, cirrhosis,
Look for signs of chronic liver disease (
dice, ascites). Feel for an abdominal mass. Listen for a bruit over the liver (
Tests Blood
FBC
, clotting,
though levels do not correlate with size, stage, or prognosis).
identify lesions and guide biopsy.
nant lesions. Do
biopsy
ERCP
(See p
244
.) May achieve a histological diagnosis; careful multidisciplinary
discussion is required if potentially resectable, as bleeding or seeding along the biopsy tract can occur. If the lesion could be a metastasis, find the primary, eg by
mammography, colonoscopy,
Liver metastases Signify advanced disease. Treatment and prognosis vary with the
type and extent of primary tumour. Chemotherapy may be eective (eg lymphomas,
germ cell tumours). Small, solitary metastases may be amenable to resection (eg colorectal cancer). In most, treatment is palliative.
Hepatocellular carcinoma (
of primary liver cancers; it is common in China
The pati ent Fatigue, appetite,
Causes
HBV
is the leading cause worldwide (esp. if high viral load; p
cirrhosis (alcohol, haemochromatosis,
sinensis; anabolic steroids.
MRI
; biopsy. Tre at me nt Laparoscopic resection of solitary tumours <3cm across 3yr
13
survival from
5
yr survival rate of 70%.37 Percutaneous ablation, tumour embolization (
tions. The antiunsuitable for local therapy.
hepatitis’ and
(sun- dry maize). Encourage coee consumption (risk
screen
% to 59%; but ~50% have recurrence by 3yrs.36 Liver transplant gives a
VEGF
drug sorafenib is given for advanced disease or earlier stage tumours
table
6.15
Con side r if at risk: eg all with cirrhosis; or chronic
Cholangiocarcinoma (Biliary tree cancer.) ~10% of liver primaries. Causes Flukes
p
431
);
p
268
;
PSC (
HBV; HCV; DM; N
(Clonorchis,
disease,
cites), malaise, bilirubin;
extrahepatic or perihilar.
76
that are,
% recur. Surgery: eg major hepatectomy + extrahepatic bile duct excision + caudate lobe resection + adjuvant chemotherapy.
complications include liver failure, bile leak, and
extrahepatic biliary tree, percutaneously or via
Benign tumours Haemangiomas The most common benign liver tumours. :
Often an incidental finding on
biopsy! May be part of von Hippel– Lindau syndrome; surgery if diagnosis is uncertain
(may be confused with
anabolic steroids, oral contraceptive pill; pregnancy. Only treat if symptomatic, or >
34
Haemobilia is late in
upper
GI
haemorrhage, and jaundice. It may be life- threatening.
35 5
yr cumulative risk if cirrhosis is present is 30% in Japan and 17% in
36
Operative mortality: 1.6%. Recurrence is more likely if histology showed neoplastic emboli in small vessels. Get early warning of recurrence by arranging imaging, eg if
Fibrolamellar
37
Milan criteria for liver transplantation in
38
TACE
= transarterial chemoembolization, eg with drug- eluting beads; it causes fever and abdo pain in 50%.
HCC
HCC
, which occurs in children and young adults, has a better prognosis.
6.36
table
6.14
). Primary hepatic tumours are
table
RUQ
pain ( liver capsule stretch).
p
272
) and evidence of decompensation (jaun-
LFT
, hepatitis serology, - fetoprotein ( in 50– 80% of
), eg from breast,
6.13
).
HCC
).
Imaging US or CT to
MRI
(p
726
is better at distinguishing benign from malig-
) and biopsy if cholangiocarcinoma is suspected. Liver
CT, MRI
, or marrow biopsy.
Prognosis O ften <6 mon ths.
HCC
) Primary hepatocyte neoplasia accounts for 90%
RUQ
pain, weight, jaundice, ascites, haemobilia.34 :
&
Africa (40% of cancers vs 2% in UK).
274
).
HCV
;35
PBC
); non- alcoholic fatty liver; aflatoxin; Clonorchis
AIH
(p
3- phase CT (de layed wash - out of contrast in a suspect mass);
38
TAC E
) are op-
Prevention
HBV
vaccination (
BOX
‘Vaccinating to prevent
). Don’t reuse needles. Screen blood. Aflatoxin exposure
HCC
in cirrhosis).16 6- monthly US
HBV
in A fric ans or ol der As ian s.
screening by
CA19
– 9 may b e helpful, p
278
); biliary cysts; Caroli’s
- nitroso toxins. The p atient Fever, abdominal pain (± as-
ALP
. Patholo gy Usually slow- growing. Most are distal
Management 70% inoperable at presentation. Of those
5
yr survival ~30%. Post- op
GI
bleeding. Stenting of obstructed
ERCP
(p
726
), improves quality of life.
US
(hype rechoic) or CT and don’t require treatment. Avoid
HCC
) or they are enlarging on 6- monthly US. Adenomas Causes:
. Think of bleeding into the biliary tree whenever Quincke’s triad occurs:
USA
.
AFP
>5.45mcg/ L (esp. if trend is rising).
HCC
: 1 nodule <5cm or 2– 3 nodules <3cm.
RUQ
HCC
HCC,
CXR
≈
280
≈
5
cm.
pain,
).
,
3
.
);
:1.
4
6 Gastroenterology
Table 6.
https://t.me/med1917
13
Primary liver tumours
Malignant (prognosis— regardless of
type— is poor)
HCC
Cholangiocarcinoma
Angiosarcoma
Hepatoblastoma
Fibrosarcoma
tumour (
* Simple cysts (no communication with the biliary tree) are present in 1%, but rarely cause symptoms.
:≈9
†
2nd most common benign solid lesion; hyperplastic hepatocytes around a stellate scar, possibly a
response to an anomalous vessel. Solitary lesion,
‡
GIST
from the muscularis propria, eg with
Table 6.
&
hepatic gastrointestinal stromal
‡
GIST
, formerly leiomyosarcoma)
:1 if large or symptomatic.
s are mesenchymal tumours that are more likely to be found in the gut as a spherical mass arising
14
Origins of secondary liver tumours
GI
bleed ing. If unre sectable, imatinib 2yr survival from 26% to 76%.
Benign
Cysts*
Haemangioma
Adenoma
Focal nodular hyperplasia
Fibroma
Benign
90
% found in .
GIST
(= leiomyoma)
†
Common in men Common in women Less common (either sex)
Stomach
Lung
Colon
Breast
Colon
Stomach
Uterus
Pancreas
Leukaemia
Lymphoma
Carcinoid tumours
Vaccinating to prevent hepatitis B (and associated complications)
Use hepatitis B vaccine 1mL into deltoid; repeat at 1 & 6 months (child: 0.5mL ≈ 3
into the anterolateral thigh).
‘low’ endemicity— in
protection against
targeting at- risk groups (
further doses. Serology helps time boosters and finds non- responders (correlates
with older age, smoking, and sex).
Table 6.
15
Post- immunization anti- HBs titres and actions
Indications Everyone (
2014
this meant that 82% of the world’s children received
HBV
). This contrasts with the approach in, eg the UK and
p
274
). The immunocompromised and others may need
Know your own antibody level!
WHO
advice, even in areas of
USA
Anti- HBs (IU/ L) Actions and comments (advice diers in some areas)
>
1000
100– 1000
<
NB
: protection begins some weeks after dose 1, so it won’t work if exposure is recent;
here, specific anti- hepatitis
Good level of immunity; retest in ~4yrs
Good level of immunity; if level approaches
Inadequate; give booster and retest
100
10
Non- responder; give another set of 3 vaccin ations. Re test; if <10 get
<
consent to check hepatitis
HB
core + ve represents past infection and immunity. If a non-
antiresponder is deem ed susceptible to
tact with risky bodily fluids, oer
B
immunoglobulin is best if not already immunized.
B
statu s: HBsAg + ve means chronic infection;
HBV
, and has recently come in con-
2
doses of anti- hep B immun oglobuli n
100
, retest in 1yr
283
of
Fig 6.
36
Axial CT of the liver after
IV
contrast showing multiple round
lesions of varying size, highly suggestive of hepatic metastases.
Courtesy of Norwich Radiology Dept.
6 Gastroenterology
Hereditary haemochromatosis (HH)
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284
An autosomal recessive disorder of iron metabolism in which intestinal iron absorption leads to iron deposition in joints, liver, heart, pancreas, pituitary, adrenals, and
skin. Middle- aged men are more frequently and severely aected than women, in
whom the disease tends to present
Genetics
HH
is one of the commonest inherited conditions in those of Northern European (es-
pecially Celtic) ancestry (carrier rate of
~
1
in
200– 400
). The gene responsible for most HH is
C282Y
and
are termed
1– 3
%, with compound heterozygotes accounting for 4– 7%. Penetrance is variable—
a significant fraction of
H63D. C282Y
C282Y
~
10
yrs later (menstrual blood loss is protective).
~
1
in 10 and a frequency of homozygosity of
HFE
: the 2 commonest mutations
accounts for 60– 90% of HH, and
homozygotes will not develop signs of iron overload
during follow- up, complicating screening decisions.
The patient
Nil— or tiredness; arthralgia (2nd + 3rd
Early on
Later Slate- grey skin pigmentation; signs of chronic liver disease (p
megaly; cirrhosis (esp. if drinks alcohol); dilated cardiomyopathy.
DM
(‘bronze diabetes’ from iron deposition in pancreas); hypogonadism (p
MCP
joints + knee pseudogout); libido.
Endocrinopathies
pituitary dysfunction.
Tests
LFT
Blood
transferrin saturation
Images Chondrocalcinosis (fig
, ferritin ( >
Perl’s stain quantifies iron loading
200
/ >
150
39
should all trigger suspicion. Confirm by
ng/ mL; but inflammation will also ferritin);
6.37
). Liver & cardiac
40
and assesses disease severity.
MRI
: Fe overload. Liver biopsy
Management
~
0.5– 2
Venesect
continue to accumulate, so maintenance venesection is needed for life (
2– 3
months to maintain haematocrit <0.5, ferritin <
saturation <
units/ 1– 2wks, until ferritin 50mcg/ L (may take 2yrs). Iron will
100
40
%). No randomized evidence, but survival benefit has been shown
mcg/ L, and transferrin
in observational studies. Consider desferrioxamine, an iron chelator (
tolerant of this.
Monitor
LFT
section the time available for Hb glycosylation. If cirrhotic, screen for
ultrasound ±
AFP
twice- yearly.
and glucose/ diabetes (p
200
). HbA1c levels may be falsely low as vene-
Over- the- counter drugs Ensure vitamin preparations contain no iron.
Diet A well- balanced diet should be encouraged— there is no need to avoid iron-
rich foods. Avoid alcohol. Avoid uncooked seafood (may contain bacteria that
thrive on increased plasma iron concentrations, eg Listeria monocytogenes, Vibrio
vulnificus).
Screening Serum ferritin, transferrin saturation, and
degree relatives by genetic testing even if they are asymptomatic and have normal
LFT
ideally prior to age where significant iron deposition likely to have occurred (eg
18– 30
yrs). Since
C282Y
homozygotes may never develop iron overload, population
HFE
genotype. Screen 1st-
screening should not be performed.
Prognosis
Venesection returns life expectancy to normal if non- diabetic and non- cirrhotic
(and liver histology can improve). Arthropathy may improve or worsen. Gonadal
failure may reverse in younger men.
cancer, especially if: age >
(risk ×
2
).
50
yrs (risk ×13), HBsAg + ve (risk ×5), or alcohol abuse
If cirrhosis, 22– 30% get hepatocellular
H63D
accounts for
276
HFE
genotyping.
p
); hepato-
226
) from
1U every
338
), if in-
HCC
with
39
Tran sf er ri n s at ura ti on >45% is a sensitive threshold for further screening but will lead to some false + ves.
40
Although generally not required, biopsy quantifies hepatic iron loading and fibrosis. This helps deter-
mine the severity of liver disease, particularly in those with other underlying causes of chronic liver disease.
6 Gastroenterology
A bit about iron metabolism
https://t.me/med1917
60
% of body iron is in haemoglobin, and erythropoiesis requires ~5– 30mg iron/
day— provided by macrophages (recycling of haeme iron after phagocytosis of old
RBC
S). Intestinal iron absorption (
Red meats, liver, seafoods, enriched breakfast cereals and pulses, and some
spices (eg paprika) are iron- rich. Most dietary iron is Fe
low gastric pH and ascorbic acid (vitamin
1– 2
mg/ day) compensates for daily iron losses.
3
+
, which is reduced by
C
) to better- absorbed Fe
2
+
. Absorption
occurs mainly in the duodenum and jejunum, though very small amounts are absorbed in the stomach and ileum. Iron requirements are greater for women (menstrual loss), when growing, in pregnancy, and in chronic infection.
Hepcidin, a peptide synthesized in hepatocytes, secreted in plasma, is a negative regulator of gut iron absorption and haeme iron recycling by macrophages.
Hepcidin synthesis is stimulated by iron and repressed by iron deficiency and by
marrow erythropoiesis (eg in anaemia, bleeding, haemolysis, dyserythropoiesis,
or erythropoietin injections). Defects in the normal triggering of hepcidin by
iron excess is a rare cause of haemochromatosis unrelated to
whereas a defect in hepcidin repression is responsible for an iron refractory iron
HFE
mutations,
deficiency anaemia. More commonly excessive dosing with oral iron will lead to
upregulation of hepcidin and reduce iron absorption— once daily or even alternate
day oral iron replacement is now the preferred strategy for patients requiring iron
supplementation.
In HH, the total body iron is up to 10- fold that of a normal person, with loading
found particularly in the liver and pancreas (≈100
17
). Hepatic disease classically
starts with fibrosis, progressing to cirrhosis as a late feature.
285
Fig 6.
37
Haemochromatosis causes stressed joints to deteriorate faster than resting joints: the
2
nd and 3rd
MCP
(right image) in this man who only used his dominant hand for his production line job.
joints have osteophytes and narrowed joint spaces compared to the normal hand
Reproduced from Watts et al., Oxford Desk Reference: Rheumatology,
2009
, with permission from Oxford University Press.
6 Gastroenterology
1- antitrypsin (
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286
A1AT
deficiency is an inherited disorder aecting lung (emphysema) and liver
(cirrhosis and
inhibitors made in the liver that control inflammatory cascades. Deficiency is
serpinopathy. It makes up 90% of serum 1- globulin on electrophoresis
called a
p
679
).
A1AT
(
deficiency is the chief genetic cause of liver disease in children. In
adults, its lack is more likely to cause emphysema, and all those with persistent airflow obstruction should be tested. Lung
neutrophil elastase— a process that is also induced by cigarette smoking (
A1AT
) deficiency
HCC
).
A1AT
is a glycoprotein and one of a family of serine protease
A1AT
protects against tissue damage from
Prevalence
~
1:4000
(higher in Caucasians).
Genetics
Autosomal co- dominant. Genetic variants of
M
bility as medium (
amino acid substitutions and result in production of
Z
= 15%). The normal genotype is PiMM, the high- risk homozygote is PiZZ; heterozy-
gotes are
), slow (S), or very slow (Z). S and Z types are due to single
PiMZ
and PiSZ (at low risk of developing liver disease).
A1AT
are typed by electrophoretic mo-
- antitrypsin (S = 60%,
1
The patient
Symptomatic patients usually have the PiZZ genotype: dyspnoea from emphysema;
cirrhosis; cholestatic jaundice. Cholestasis often remits in adolescence.
Tests
Serum
A1AT
levels , usually (eg <11µmol/ L or <75% of lower limit of normal, which
~
0.9
g/ L; labs vary). Note the ‘usually’. Because
is
A1AT
is part of the acute- phase
response, inflammation may hide a low level. Unless you do genotyping, you will
inevitably mislabel some cirrhosis as cryptogenic.
Lung function testing Shows reductions in
There may be some bronchodilator reversibility.
Liver biopsy (See p
244
.) Periodic acid Schi (
FEV
with obstructive pattern (p
1
PAS
) + ve; diastase- resistant globules.
Phenotyping By isoelectric focusing requires expertise to distinguish SZ and ZZ
phenotypes. Phenotyping can miss null phenotypes.
Prenatal diagnosis Possible by
11– 13
wks’ gestation.
Management
Smoking cessation, avoid passive exposure. Prompt treatment/ preventative vaccination for lung infections. Giving
COPD
exacerbations may be prevented (no good randomized trials).
but
DNA
analysis of chorionic villus samples obtained at
IV A1AT
pooled from human plasma is expensive
Liver transplantation Needed in decompensated cirrhosis.
Lung transplantation Improves survival and has a comparable survival to trans-
A1AT
- deficient
COPD
plantation in non-
Inhaled
A1AT
Has been tried in lung disease.
.
Prognosis
Some patients have life- threatening symptoms in childhood, whereas others remain asymptomatic and healthy into old age. Worse prognosis if male, a smoker,
or obese. Emphysema is the cause of death in most, liver disease in
cirrhosis ±
HCC
aect 25% of
A1AT
- deficient adults >50yrs.
p
166
~
5
%. In adults,
).
154
).
6 Gastroenterology
Approaches to abnormal liver function tests (
AST, ALT
GGT
AIH
OHCS
https://t.me/med1917
Abnormal
Also, remember that normal
LFT
S can be found in ~
17
% of the asymptomatic general population.
LFT
S do not exclude liver disease.
LFT
S)
Te st s of h e p at oc e l lu l ar i n ju r y o r ch o le s ta s is
Aminotransferases (
ALT
injury.
and muscle).
is more specific for hepatocellular injury (but also expressed in kidney
AST
is also expressed in the heart, skeletal muscle, and
): released in the bloodstream after hepatocellular
RBC
S.
Alkaline phosphatase: may originate from liver, bone (so raised in growing chil-
dren) or placenta; isoenzyme testing (where available) may dierentiate source.
Gamma- glutamyl transferase (
and intestine— but not bone, so it helps tell if a raised
GGT
). NB: it is not specific to alcohol damage to the liver.
liver (
; γGT): present in liver, pancreas, renal tubules,
ALP
is from bone (
Tests of hepatic function
Serum albumin, serum bilirubin, PT (
INR
).
Hepatocellular predominant liver injury
AST &
ALT
family (‘Could he be consuming alcohol?’); ultrasound for fatty liver, metastases,
viral serology (hepatitis
Alcoholic liver disease:
not reliable, normal
Acute viral hepatitis:
Chronic viral hepatitis:
Autoimmune hepatitis (
Fatty infiltration of the liver: (see p
LFT
. Evaluate promptly, consider medications, collateral history from
A, B, C, E, EBV, CMV
AST/ ALT
ALP
,
GGT
, and macrocytosis suggest this condition.
ALT
; bilirubin may be . NB:
ALT; HBV & HCV
): occurs mainly in young and middle- aged females.
S in the general population and may be recognized on ultrasound.
).
ratio is typically 2:1 or more. When the history is
AST
may be , p
are a leading cause worldwide.
281
.) Probably the chief cause of mildly raised
274
, p
Ischaemic hepatitis: can be seen in conditions when eective circulatory volume
MI
is low (eg
, hypotension, haemorrhage).
ALT
, as well as
LDH
.
Drug- induced hepatitis: as no specific serology identifies most culprits, a good
history is vital. Paracetamol overdose causes most acute liver failure in the
Cholestasis predominant liver injury
ALP
and
GGT
are ;
AST
and
ALT
mildly .
Management
For each specific diagnosis, manage accordingly. If asymptomatic and other tests
are −ve, try lifestyle modification. Help reduce weight and alcohol use (
p
826
); control DM & dyslipidaemia; stop hepatotoxic drugs.
Fol low- up
Repeat tests after 1– 2 months; if still , do US (± abdominal CT). If diagnosis still
unclear, get help: is biopsy needed? Consider (if you haven’t already)
serum caeruloplasmin (Wilson’s disease), coeliac serology,
p
280
).
ANA
, and
A1AT
ASMA (AIH
GGT
277
) or
.
UK
.
p
276
&
levels,
287
,
7
https://t.me/med1917
Kidney medicine
Contents
Urine
290
Urinary tract infection (
Acute kidney injury (
A clinical approach
Management
Chronic kidney disease (
Management
Renal replacement therapy (
Dialysis and filtration
Transplantation
Glomerulonephritis
Nephrotic syndrome
Systemic disease in the kidney
Kidney tubules: disorders and
diuretics
Tubulointerstitial nephropathy and
Inherited kidney disease
312
nephrotoxins
296
300
314
304
AKI
306
308
294
UTI
):
CKD
302
316
)
292
)
298
RRT
):
310
Fig 7.
1
It was not adequate for Ronald and Richard
Herrick to tell the world that they were identical twins.
17
formal genetic tests were undertaken, as
Instead,
well as an examination of their fingerprints, coordinated at a local police station, witnessed by journalists.
When a reciprocal skin graft, in the absence of any
immunosuppressive armoury, remained well healed
it was declared that 'the probability of identity was
excellent'. During this time, Richard was dying of
kidney failure. He was erratic, uncooperative, and
disorientated. The psychiatric evaluation concluded
uraemic encephalopathy: 'Ohand, I feel the patient
will recover from his psychosis with... removal of toxic
agents.’ He was hypertensive and fluid overloaded
with primitive dialysis leading to uncontrolled electrolyte shifts and arrhythmias. But would Ronald's
kidney fit inside Richard? A test run was required to
ensure anatomy and logistics did not compromise
the planned surgery. On
Murray abandoned his preparation of Christmas
75
eggnog for
room where a cadaver had finally become available for
practice. Three days later the first successful human
kidney transplant was performed. Although advances
in dialysis, surgery, and immunosuppression over the
intervening
those with kidney transplants, the bravery of living
donors remains. Richard knew this: 'Get out of here
and go home', he wrote on the eve of the surgery. 'I
am here and I am going to stay', was Ronald's reply.
Ronald not only gifted a kidney to Richard, but also
days he should not have had, a wife he had not yet met,
and two children who glomerulonephritis had decreed
should not have been born.
20
guests and travelled to a post- mortem
70
years have transformed the lives of
December
1954
, Dr Joseph
Artwork by Gillian Turner.
We thank Andrew Mooney, our Specialist Reader for this chapter.
7 Kidney medicine
Kidney disease presents as:
CKD
NSAID
https://t.me/med1917
1
Asymptomatic disease
• Non- visible haematuria (
stick. Confirm with repeat testing. Most is not due to kidney disease. Urological
investigation is first line for all aged >
• Asymptomatic proteinuria Normal kidney protein excretion is <
pregnant). Quantification by
ical practice. Spot urinary albumin to creatinine ratio (u
mmol, or urinary protein to creatinine ratio (u
erular (common) or tubular (rare) pathology.
• Abnormal kidney function (
of how much blood the kidneys are cleaning per minute. Direct measurement is
invasive and time- consuming. Estimates derived from equations based on serum
creatinine are widely used to give an e
non- steady- state conditions (eg
(diet, muscle mass). e
which require other evidence of kidney disease.
• High blood pressure Kidney disease should be excluded if hypertension occurs
with any of haematuria, proteinuria, e
• Electrolyte abnormalities Disorders of sodium, potassium, and acid– base balance
p
297
, pp
662–7
(
2
Kidney tract symptoms
) may be due to underlying kidney disease.
NVH
, microscopic haematuria) Detected on urine dip-
40
yea rs. Se e p
24
h urine collection is unreliable and not used in clin-
GFR
) The g lomer ular filtration rate (
GFR
AKI
GFR
is le ss accurate at hi gher leve ls of
), and conditions which alter serum creatinine
GFR
290
.
ACR
PCR
(see p
) >2.5() or 3.5()mg/
) >15mg/ mmol may signify glom-
661
). Errors in e
GFR
.
150
mg/ 24h (non-
GFR
) is a measure
GFR
are caused by
(>60mL/ min/ 1.73m2),
• Urinary symptoms Dysuria is a sensation of discomfort with micturition and
may be accompanied by urgency, frequency, and nocturia. Urinary tract infec-
(UTI)
is the primary dierential. Consider prostatic aetiology if there is dif-
tion
ficulty initiating voiding, poor stream, and dribbling. Oliguria (<
0.5
mL/ kg/ h) and anuria should trigger assessment and investigation for acute
<
kidney injury (
volumes of urine, usually from high fluid intake but consider also
insipidus (
concentration of urine).
• Loin pain For pain confined to the loin consider pyelonephritis, kidney cyst path-
ology, and kidney infarct. Ureteric colic is severe and radiates anteriorly from the
loin to the groin. It can be caused by caliculi, clot, or a sloughed papilla.
• Visible haematuria (
clude renal tract malignancy. Nephrological causes include polycystic kidney disease and glomerular disease:
(anti-
• Nephrotic syndrome Proteinuria >
hypoalbuminaemia (<
• Symptomatic chronic kidney disease (
pruritus, bone pain, sexual dysfunction, cognitive decline (
3
A systemic disorder with kidney involvement
• DM (Se e p
• Metabolic Si ckle c ell d isea se (p
cystinosis (
• Immune- mediated
Schönlein purpura (
drome (
• Infection Sepsis is a common cause of
clude
• Malignancy Obs truct ion, h yperca lcaem ia, di rect tox icity, eg myel oma (p
• Pregnancy Pre- eclampsia.
• Medication
AKI
) (see pp
p
234
), hypercalcaemia (p
VH
GBM
) disease (p
307
30
310
.)
p
317
).
ANCA
p
p
314
TB
(p
307
, p
548
).
388
), malaria, chronic hepatitis (p
s, aminoglycosides, chemotherapy.
294–5
). Polyuria is the voiding of abnormally high
668
), renal medullary disorders (impaired
, macroscopic) Urological investigation is required to ex-
IgA
(p
307
), Alport syndrome (p
), anti- glomerular basement membrane
316
).
3
g/ 24h (= u
PCR
g/ L) and oedema. Kidney biopsy is usual in adults (p
) Dyspnoea, anorexia, weight loss,
311
), tuberous sclerosis (p
- associated vasculitis (p
), systemic sclerosis (p
310
, p
554
311
), sarcoid (p
AKI
. Systemic infections leading to
274
),
HIV
(pp
394– 9
ACE-
kidney function by reducing intraglomerular pressure. They may exacerbate
kidney perfusion is reduced, eg sepsis, volume depletion, but remember to re- start
ACE-
i/
ARB
fol lowing acute illne ss.
400
DM,
>
300
mg/ mmol) with
pp
298–301
).
316
), Fabry disease (p
),
SLE
(p
310
314
), Sjögren’s syn-
)
.
i,
ARB
are used to preserve
mL/ 24h or
diabetes
306
).
316
), Henoch–
CKD
in-
310
).
AKI
289
),
if
7 Kidney medicine
Urine
https://t.me/med1917
290
Perform dipstick urinalysis whenever you suspect kidney disease. This is a quick
way of checking whether the urine contains anything that it should not, eg protein,
blood, glucose. Abnormalities can indicate intrinsic kidney disease or kidney tract
abnormalities and usually require further investigation.
A dipstick test of a catheter sample is dicult to interpret.
Look for a urine dip result (before the catheter was inserted). A positive result
may need specialist advice from nephrology/ urology. A negative result may help to
reassure you about the absence of intrinsic kidney disease.
Proteinuria
Requires quantification. 24h collection of urine is not used due to inaccuracy. Urinary
albumin:creatinine ratio (u
rando m spot urine sampl e. Normal u
PCR
<15. Approximate equivalent levels of proteinuria are given in table
than u
Table 7.
1
Conversion factors
Protein excretion g/ 24h
0.15
(physiological)
0
.
5 30 50
1 70 100
3
(nephrotic range)
Raised u
ACR/ uPCR
The higher the proteinuria, the greater the chance of glomerular
disease, eg glomerulonephritis,
of cardiovascular disease and death.
morning sample), post- exercise, fever, heart failure. in pregnancy: u
ACR <8
and u
mmg/ mmol are considered normal for pregnancy.
Microalbuminuria Ultra- sensitive dipsticks are available to measure microalbuminuria
(albumin excretion
Haematuria
Blood in the urine may arise from anywhere in the renal tract. Transient causes
should be excluded, eg
• Visible (macroscopic, frank).
• Invisible (microscopic): detected on dipstick testing or microscopy.
30– 300
Causes Malignancy (kidney, ureter, bladder), calculi, IgA nephropathy, Alport syndrome
p
316
), other glomerulonephritides (p
(
iasis. Do not attribute haematuria to anticoagulation without investigation.
False positive Myoglobin triggers dipstick reaction but microscopy will be nega-
tive for red blood cells.
Management Refer for urological assessment: imaging, and cystoscopy, to exclude
renal tract malignancy/ calculi if aged
and referral to nephrology for invisible haematuria with:
• e
GFR
<60mL/ min/ 1.73m2.
• Coexistent proteinuria (u
• Hypertension >
• Family history of kidney disease.
No cause is found in
monitor
GFR
warrant referral to nephrology.
e
BP
, e
140/ 90
GFR
, and repeat u
Others
DM
Glucose
Starvation, ketoacidosis.
teric Gram−ve organism).
olysis.
, pregnancy, sepsis, proximal renal tubular pathology (p
Specific gravity Normal range: 1.
aected by proteinuria.
diet (acid– base balance,
ACR
) or protein:creatinine ratio (u
ACR
is <2.5() or <3.5() and is more sensitive
UACR
mg/ mmol
2.5
or 3.5
250 300
DM
, amyloidosis. Proteinuria is associated with risk
PCR
) is performed on a
UPCR
mg/ mmol
15
7. 1
.
False positive Postural (repeat using an early
PCR
<30mg/ mmol
mg/ 24h). Suggests early glomerular disease, eg DM.
UTI
, menstruation. Classified as:
306
), polycystic kidney disease (p
45
ACR
>30mg/ mmol or u
or over. Consider intrinsic kidney disease
PCR
>50mg/ mmol).
316
), schistosom-
mmHg.
19– 68
% of invisible haematuria. If there is no proteinuria,
ACR
/ u
PCR
annually. Proteinuria and/ or deteriorating
312
Leucocytes
Bilirubin Haemolysis. Urobilinogen Liver disease, haem-
UTI (
p
292), vaginal discharge. Nitrites
005– 1.030
, surrogate for urine osmolality,
). Ketones
UTI
(en-
pH Normal range: 4.5– 9, usually acidic with meat- containing
p
662
; renal tubular acidosis, pp
312– 13
).
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