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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_47_библиотеки_им_акад_М_И_Перельмана
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patient may have the liver inltrated by mycobacteria, for example [61]. Patients
with renal insufciency from polycystic kidney disease may have liver cysts as part
of the syndrome [62]. Stauffer’s syndrome, characterized by hepatomegaly and cho-
lestasis in the absence of liver metastases, can be a manifestation of patients with
renal cell carcinoma; the syndrome resolves when the tumor is resected [63]. Cystic
brosis can be associated with cholestasis [64]. Subtle changes in the liver prole,
including increased activity of transaminases, can be seen in hypothyroidism and
hyperthyroidism. Basedowiana-Haban cirrhosis, characterized by hepatomegaly
and episodes of jaundice, was originally described in patients with thyrotoxicosis by
the German physician Karl Basedow [65, 66]. In the context of interpreting thyroid
function tests, it is important to recognize that an increase in thyroid binding globu-
lin, and not hyperthyroidism, appears to be the most common cause of increased
serum levels of thyroxine (T4) in patients with liver disease [67]. Free T4 concentra-
tions are necessary to conrm hyperthyroidism [68].
Past Medical andSurgical History
A history of jaundice or abnormal liver prole may suggest previous hepatitis or the
passage of a gallstone, sometimes accompanied by pain. History of gallbladder sur-
gery, including laparoscopic cholecystectomy, in a patient with cholestasis, should
trigger the search for post-surgical biliary strictures, which need to be repaired to
prevent biliary cirrhosis [69]. Biliary strictures can also be a complication of chronic
pancreatitis [70].
Family History
A history of liver disease in family members is relevant as it may be lead to the
diagnosis of hereditary diseases, including primary hemochromatosis [71], Wilson’s
disease [71], and alpha-1-antitrypsin deciency [72]. History of viral hepatitis B in
the mother may suggest perinatally acquired hepatitis in the patient under evaluation.
Review ofSystems
A orid presentation of hemochromatosis may include arthralgias and glucose
intolerance, hypogonadism, and cardiomyopathy. Psychiatric and neurological
alterations may suggest Wilson’s disease. Classically, emphysema and liver disease
suggest alpha-1-antitrypsin deciency; liver disease may present without
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pulmonary complications in this condition. Easy bruising in a patient with inltra-
tive liver disease suggests amyloidosis, and in a patient with “red eyes,” silvery skin
nodules, and pulmonary complaints, it suggests sarcoidosis [73]. Dyspnea may sug-
gest hepatopulmonary syndrome. CREST (calcinosis, Raynaud’s phenomenon,
esophageal dysmotility, sclerodactyly, and telangiectasias) syndrome can be associ-
ated with PBC [74].
The Physical Examination
Signs of liver disease may be overt or absent. Fever in a patient with liver disease
should trigger the exclusion of infections. In patients with decompensated liver dis-
ease, the blood pressure tends to be low, secondary to systemic vasodilatation.
Fetor Hepaticus
A sweet odor emanating from the patient’s breath can be perceived in patients with
advanced liver disease, including those with extensive portosystemic shunting [75].
The Skin, Mucous Membranes, andNails
Jaundice is the yellow discoloration of the skin, conjunctivae, and mucous mem-
branes. It jumps out at the examiner (Fig.2.1) [76, 77]. It is a manifestation of liver
disease, secondary to intra- or extrahepatic cholestasis, i.e., from acute hepatitis,
hepatocellular failure, or biliary tract obstruction. Excess in bilirubin production
from hemolysis is also a cause of jaundice. Kayser–Fleischer rings, a brown rim on
the cornea’s periphery, secondary to copper accumulation in Descemet’s membrane,
is a classic nding of Wilson’s disease [78, 79]; it is necessary to examine the cor-
nea with a slit lamp for adequate visualization most of the times. In cases of pro-
longed cholestasis and not related to Wilson’s disease, Kayser–Fleischer rings may
also be present.
The skin of patients with liver disease and pruritus does not display pruritic skin
lesions, but excoriations complicated by prurigo nodularis may abound, especially
in patients with severe pruritus (Fig.2.2); they are evidence of how devastating this
symptom can be. The skin is warm from the hyperdynamic circulation of patients
with portal hypertension. Spider angiomata are characterized by vascular arboriza-
tions that blanch on pressure (Fig.2.3); they are found on the face and upper back,
thorax, and upper arms [80]. The pathogenesis of spider angiomata has been
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considered to be systemic excess of estrogen and portosystemic shunting from cir-
rhosis. Skin hyperpigmentation is common in patients with cholestasis, in particu-
lar, that secondary to PBC.Patients with PBC may display what has been named the
“buttery sign,” an area of relative hypopigmentation between the scapulae in rela-
tionship to the surrounding skin (Fig.2.4). A gray to brown discoloration of the skin
and mucous membranes due to the accumulation of hemosiderin and hemofuscin
(gray hue) and melanin (brown hue) suggests hereditary hemochromatosis [81].
Fig. 2.2 Prurigo lesions in
a patient with chronic
pruritus from cholestasis
Fig. 2.3 Spider angiomata
on the right side of the
neck in a woman with
decompensated liver
disease in association with
alcohol use disorder
Fig. 2.4 Buttery sign on
the back of a patient with
primary biliary cholangitis
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Xanthomatosis, accumulation of lipids in the skin, is a manifestation of hyperlipid-
emia in cholestasis (e.g., PBC); it presents as plane xanthomata on the palms, soles,
trunk, and exor surfaces, as tuberous xanthomata over the joints, and as xanthelas-
mas on the eyelids and under the eyes [33, 82]. Lichen planus is also associated with
PBC. “Paper money” skin, characterized by telangiectasias on the cheeks, can be a
sign of cirrhosis (Fig.2.5) [83]. Purpura is a manifestation of vasculitis, which can
be associated with chronic hepatitis C and B infections [84]. Telangiectasias on lips
suggest CREST syndrome or hereditary hemorrhagic telangiectasia, in which vas-
cular malformations can occur in the liver [85]. The distribution of body hair of men
with cirrhosis is feminine. Nails can display white horizontal lines, Muerhcke’s
lines, indicative of hypoalbuminemia. Azure lunulae (sky blue moon) discoloration
of the nails [86] and a green hue on the skin from the accumulation of copper are
described in Wilson’s disease.
Glands andLymph Nodes
Parotid gland enlargement can be detected in 20% of patients with cirrhosis second-
ary to alcohol abuse [87] (Fig. 2.6). Testicular atrophy and feminization [88],
including gynecomastia [89], is a classic nding of men with cirrhosis, which may
result from increased peripheral conversion of androgens; tender gynecomastia is
also a side effect of spironolactone, a diuretic used to treat ascites [90].
Fig. 2.5 Paper money
appearance in a patient
with decompensated liver
disease from alcohol abuse
disorder
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Cardiovascular System
Findings suggesting pericardial (e.g., constrictive pericarditis) or cardiac disease,
including cardiomegaly, gallops, and distended neck veins, may explain hepato-
megaly and ascites [91]. Pulmonary hypertension associated with portal hyperten-
sion or liver disease (i.e., portopulmonary hypertension) is suggested by an
accentuated pulmonary component of the second heart sound at cardiac auscultation
and the presence of a systolic murmur consistent with tricuspid insufciency [92].
Pounding pulses from systemic vasodilatation and increased cardiac output are
characteristic of patients with decompensated liver disease [93].
Respiratory System
Decreased breath sounds at the lung bases, usually at the right base, suggest hydro-
thorax in a patient with decompensated liver disease and ascites. Hepatopulmonary
syndrome, which is associated with intrapulmonary vascular dilatation, is character-
ized by orthodeoxia (i.e., arterial deoxygenation from the supine to the upright posi-
tion) and platypnea (i.e., dyspnea from the supine to the upright position) [94].
These phenomena result from increases in intrapulmonary shunting due to perfu-
sion of the lower lobes [94].
Fig. 2.6 Parotid glad
enlargement in a patient
with cirrhosis in
association with alcohol
use disorder
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Abdomen
Dilated veins on the abdominal wall may be a sign of intrahepatic portal hyperten-
sion (Fig.2.7). This phenomenon results from the recanalization of the paraumbili-
cal or umbilical veins to decompress the portal system. Veins that radiate away from
the umbilicus become prominent and are described as the “caput Medusa,” after the
gorgon of Greek mythology with snakes entwined in her hair. Dilated abdominal
veins can also be seen high up in the abdomen.
In patients with liver disease, abdominal auscultation may reveal a venous hum,
suggesting portal hypertension; a bruit over the liver area, in synchrony with the
pulse, suggests a vascular tumor with arterial blood supply (e.g., hepatocellular car-
cinoma) or orid alcoholic hepatitis. A hepatic friction rub has been described in
tumors eroding the liver capsule, hepatic syphilis, and liver abscesses. Distended
abdomen and bulging anks suggest ascites (Fig.2.8), a dening complication in
the natural history of liver disease, as it correlates with morbidity and mortality.
Ascites can be detected by palpation and percussion, seeking a uid wave and shift-
ing dullness. Abdominal tenderness in a patient with ascites suggests peritonitis
(e.g., spontaneous bacterial peritonitis), which is an ominous complication of liver
disease.
The liver is dull to percussion. To feel the liver, the abdomen is examined with
the patient in the supine position, arms parallel to the sides of the body, and the
knees bent, to relax the abdominal muscles. It is useful to start palpating from the
right lower quadrant of the abdomen toward the ribcage, to encounter the liver edge
on the way up. The liver edge is smooth, and sometimes a little tender when pressed.
In general, feeling the edge up to 2cm below the right costal margin is considered
normal. The liver can be displaced downwards by emphysematous lungs. In thin
Fig. 2.7 Dilated veins
(arrows) in the abdominal
wall in a patient with portal
hypertension and
decompensated liver
disease as evidenced by
ascites (arrow head:
epigastrium)
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subjects, the liver can be felt on deep inspiration, not necessarily suggesting hepatic
pathology. Hepatomegaly may indicate cirrhosis, inltrative disease, or space-
occupying lesions (e.g., tumors). A hard consistency of the liver is consistent with
cirrhosis [95]. In disease, the liver can extend over the midline, and the left lobe can
be felt in the epigastrium. When hepatomegaly is uncertain, the scratch test may be
useful. This maneuver is conducted by placing the bell of the stethoscope on the
right upper quadrant over the ribcage and scratching the abdominal wall’s surface
from the mid-abdomen toward the liver. When the scratch is done on an area under
which the liver lays, the sound is amplied. When there is ascites, the liver edge can
be made to bounce by exerting a quick pressure with the ngertips below the rib-
cage. Splenomegaly suggests portal hypertension.
Fig. 2.8 Abdominal
distension from ascites in a
patient with
decompensated liver
disease from alcohol abuse
disorder
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Extremities
Red palms, especially on the thenar and hypothenar eminences, can be seen in
patients with cirrhosis (Fig.2.9). Retraction of the palmar fascia with subsequent
contracture of palms and ngers is known as Dupuytren’s contracture (Fig.2.10),
with increased prevalence in patients with liver disease. Acquired nger clubbing
can be seen in patients with cirrhosis and patients with hepatopulmonary syndrome
(Fig.2.11) [96].
Fig. 2.9 Palmar erythema
in the thenar and
hypothenar eminences in a
patient with cirrhosis from
chronic hepatitis C
Fig. 2.10 Incipient
Dupotryen’s contracture
(arrow) and palmar
erythema (short arrow) in a
man with cirrhosis from
alcohol abuse disorder
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Lower extremity edema complicates uid retention in liver disease. The edema
is usually pitting unless it is chronic or complicated by venous insufciency, in
which the appearance can be reminiscent of elephantiasis.
Neuropsychiatric Alterations
Family members can report subtle alterations in personality. Constructional apraxia
(e.g., inability to draw a ve-point star or to write legibly) in fully conscious patients
is a nding of hepatic encephalopathy. The Reitan number connection test can be
used to diagnose and follow the response to treatment of hepatic encephalopathy.
Asterixis, a sign of encephalopathy, can be appreciated by having the patient extend
the arms, palms down, and dorsiex the wrists while separating the ngers for at
least 15s. It is characterized by a series of extension and exion movements at the
level of the wrist. Electromyography of the contracted muscles has revealed lapses
of electrical input that coincide with asterixis [97]. Tremors can also be noted.
Investigation ofLiver Disease
An impression of liver disease can be conrmed by laboratory exams, radiographic
tests, or a combination, which should be analyzed in concert. A liver prole sugges-
tive of cholestasis, including that resulting from inltrative disease, is characterized
by an increase in alkaline phosphatase serum activity and, in the majority of cases,
gamma-glutamyl transpeptidase. Transaminases are reportedly released by injury to
the hepatocytes, such as that occurring in acute or chronic hepatitis. A prolonged
prothrombin time (or high International Normalized Ratio) suggests synthetic
Fig. 2.11 Finger clubbing
in a patient with
hepatopulmonary
syndrome associated with
cirrhosis secondary to
alcohol use disorder
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hepatic dysfunction, as does a decreased concentration of serum albumin. A com-
plete blood count may reveal pancytopenia secondary to bone marrow suppression
and hypersplenism due to portal hypertension, which tends to be associated with
thrombocytopenia.
The review of the urine analysis and renal function tests in patients with liver
disease is very relevant. Bilirubinuria is a sign of liver damage and usually precedes
jaundice. In the absence of bilirubinuria, jaundice is from unconjugated hyperbili-
rubinemia (e.g., from hemolysis) [98]. Microscopic hematuria and proteinuria sug-
gest glomerulopathies (e.g., associated with cryoglobulinemia, deposition of
immune complexes, most commonly IgA). An alkaline urine pH suggests renal
tubular acidosis, which can complicate Wilson’s disease and PBC.Renal insuf-
ciency, oliguria, and marked reduction of urine sodium are consistent with hepatore-
nal syndrome, a dreadful complication of liver disease.
A liver sonogram with Doppler studies is an important tool in the investigation
of liver disease. This test gives information on liver architecture, vascular ow, bili-
ary obstruction or bile duct dilation, gallbladder disease, and echogenicity that may
suggest liver disease, fat, and space-occupying lesions, including livers cysts, and
masses [99]. A sonographic study of the liver can also suggest parenchymal liver
disease (e.g., increased echogenicity, nodularity) and the opportunity to identify
space-occupying lesions, including tumors and cysts. Other radiological modalities
include computed tomography, usually with contrast, if the renal function allows it,
multiphasic, or magnetic resonance imaging, especially for the characterization of
liver masses, including hepatocellular carcinoma [100, 101].
Liver Biopsy
To examine the liver histology provides the opportunity to diagnose or conrm a
particular disease, determine the degree of liver injury (e.g., presence of cirrhosis)
and assess the response to treatment. The decision to perform a liver biopsy is
always individualized, and the risks versus benets are considered.
Noninvasive methods to determine liver brosis are available, including
Fibrosure, Fibrotest-Actitest, and the aspartate transaminase to platelets ratio index
(APRI). They combine the analysis of certain blood components including serum
proteins and enzyme activities and for the APRI, platelet count, to generate a score
that provides information regarding inammatory activity and brosis. Transient
elastrography assesses the liver’s stiffness by measuring the velocity of a vibration
wave, i.e., shear wave, generated through an intercostal space over the liver area.
Shear wave velocity is determined by measuring the time the vibration wave takes
to travel to a particular depth inside the liver. In contrast to the normal liver, the
brotic liver is hard; thus, the degree of brosis can be inferred from liver stiffness.
The results are expressed in kilopascals (kPa) [102–104]. Concerns about the reli-
ability of these tests exist; however, the wish to develop reliable noninvasive exams
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