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The scenario suggests this woman may have bled from oesophageal varices. However, even in the presence of known varices, it must not be assumed that any upper gastrointestinal haemorrhage is due to these varices. Other causes, such as peptic ulceration and metagenic mucosal rupture (Mallory-Weiss syndrome), must also be considered. In this instance the question is not so much what has caused the bleeding, but what prognostic factors are present. Management of any acute event, including a bleed, may be tempered in the presence of indicators of poor prognosis. The hypotension on admission is a reflection of the severity of the bleed and in itself is not a prognostic sign. Hypotension resistant to any therapy might certainly suggest poor prognosis, but in this case we are not told anything about the resuscitative measures. The prognostic factors considered in the assessment of a patient with portal hypertension include both physical and biochemical parameters. Encephalopathy and ascites resistant to treatment are poor signs, as are elevated bilirubin, low serum albumin and prolonged INR (international normalised ratio) (B is correct). Together, these five criteria make up the Child-Pugh classification of severity of disease in patients with portal hypertension. Other physical signs, such as number of spider naevi and liver size, may give a clue to the underlying cause of the portal hypertension but are not used as indicators of severity of disease. Likewise, the serum alanine aminotransferase (ALT) levels are less important than the albumin and INR.
With the advent of high-resolution imaging and the increasing frequency of its use, unexpected or incidental findings are a common problem for the clinician. This case illustrates a typical scenario. The patient has symptoms that might be explained by gallstones. Has the patient experienced recurrent bouts of biliary colic? Previously, a focused examination such as an oral cholecystogram would have confined attention to the gallbladder, but with ultrasound and computed tomography (CT), not only will the targeted structure or organ be imaged, but adjacent tissues also examined. This has clear advantages in terms of definition of the chosen structure and its surrounds, but does raise problems with any unexpected and possibly irrelevant finding. Liver lesions are common and many are innocent. The CT can usually provide an exact diagnosis, but such imaging may need to be complemented by ultrasound, magnetic resonance imaging or biopsy. In this case there are two issues. First, can a diagnosis be made on the basis of the CT and, second, do the CT findings explain the patient's symptoms? Most CT examinations are performed with one or more contrast materials used to highlight particular structures (vascular, gut, urinary tract). In this case intravenous contrast has been given and image acquisition will be timed to coincide with arterial and venous flow - in addition to the observation of a contrast-free phase. The description provided is characteristic of a haemangioma (B is correct). Haemangiomas are probably the most common liver tumour and may be found in up to 20% of the general population. They may be solitary or multiple. Most are congenital, do not change size and remain asymptomatic. The lesions typically show intense enhancement during the arterial phase of CT and retain a blush of contrast during the portal venous phase. It is unlikely that this lesion was responsible for the patient's symptoms - the finding should be explained to the patient and the lesion should be left well alone. If there is any doubt to the diagnosis, a magnetic resonance imaging (MRI) scan might be performed or the CT repeated in a few months time to look for any possible changes. The other lesions provided as distractors can all surface as incidental findings on a CT. They each have their characteristic appearance. The CT appearance of metastatic liver lesions will vary according to their site of origin. They can be single or multiple. Most liver metastases are hypovascular on the non-contrast scans when compared with the surrounding parenchyma and this becomes more pronounced in the portal venous phase. In isolation, a hepatocellular carcinoma can be difficult to distinguish on CT from a metastatic deposit, but the presence of cirrhotic nodules may help in the diagnosis. Cystic lesions in the liver may be simple, multiple (polycystic disease), neoplastic or infective (hydatid, abscess). Simple cysts are extremely common and usually asymptomatic. On imaging, these cysts have a low density homogenous appearance. Polycystic disease is shown as thin-walled homogenous cysts - and the number and size of these lesions often lead to symptoms. Neoplastic cysts are rare, often multiloculated and heterogeneous. Hydatid cysts have a characteristic septate appearance and heterogeneous appearance if daughter cysts are contained within. Liver abscesses are usually symptomatic and tend to have a heterogeneous appearance. (缺图)
Small bowel tumours are not common - and are certainly much less common than tumours elsewhere in the digestive tract, particularly the large bowel. Whereas the majority of colorectal tumours are malignant, most small bowel neoplasms are benign. Of the malignancies within the small bowel, perhaps 50% will be adenocarcinoma - with carcinoids, lymphomas, metastatic deposits and gastrointestinal stromal tumours accounting for the rest. In regions of the world where malignant melanoma is prevalent, metastatic deposits of this tumour may account for a large proportion of small bowel tumours. Spontaneous primary lymphoma of the small bowel is most often found in the terminal ileum, but that associated with coeliac disease tends to occur in the proximal jejunum. All the conditions listed are risk factors for some form of gastrointestinal tract malignancy. Crohn disease is associated with a 100-fold increase in risk of developing adenocarcinoma of the diseased section of bowel - usually the distal ileum. The Peutz-Jeghers syndrome is associated with development of hamartomas, which in turn may undergo malignant transformation into adenocarcinoma. As mentioned above, coeliac disease is an important risk factor to be considered in patients found to have primary jejunal lymphoma (C is correct). Patients with familial adenomatous polyposis are at increased risk of duodenal and small bowel carcinoma and, even after total proctocolectomy, need to be kept under surveillance for the development of small bowel malignancy. Patients who have undergone gastric resection more than twenty-five years earlier (usually for benign disease) are at increased risk of developing adenocarcinoma in the gastric stump.
Her condition of familial adenomatous polyposis (FAP) is inherited as an autosomal dominant mutation with high penetrance, as illustrated. Half of her offspring of either sex are likely to be affected - a probability of 0.5 (C is correct). In FAP there is an inherited propensity to develop multiple adenomas through the colon and rectum, some of which inevitably become malignant in time. Treatment of affected patients can be by total proctocolectomy and ileostomy, or total colectomy with ileorectal anastomosis and continuing endoscopic review of the rectal segment. Half the children of affected parents are likely to inherit the parent's condition. The polyps appear at around puberty. Most affected patients have developed polyps by age 20 years. If untreated, malignant change develops earlier than the normal age range, often in the 30s to 40s. Prophylactic colonoscopic screening of all children of affected parents and total proctocolectomy or colectomy with ileorectal anastomosis and subsequent rectal surveillance are recommended early after diagnosis. Ophthalmoscopic screening for associated retinal pigmentation is also helpful. The genetic mutation is a deletion of genetic material, causing a derangement of cell formation common to many tissues, so that extracolonic manifestations can occur; and patients require additional ongoing upper gastrointestinal endoscopy for the presence of duodenal malignancies. Gardner syndrome of FAP is the most severe form of the syndrome - with associated osteomas, desmoid tumours of the abdominal wall, and sebaceous cysts. (缺图)