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Biomedical subjects

N A Collier

Publications and source records attributed to N A Collier.

10 recordsLinked to original sources

Multiple endocrine neoplasia syndrome--type 2b. Case report and review.

The multiple endocrine neoplasia syndromes are an association of tumours of 2 or more endocrine glands. Multiple endocrine neoplasia type 2b (MEN 2b) patients develop medullary thyroid carcinoma and pheochromocytomas as well as unique physical characteristics. Most commonly, MEN2b is inherited with an autosomal dominant pattern although sporadic cases are not uncommon. If untreated the disease may be lethal. The facial, oral and ocular characteristics are reliable markers of the disease. These patients give a history most commonly of slipped capital femoral epiphysis, hypertension and life-long diarrhoea and/or constipation. MEN2b is most commonly characterised by nodules on the anterior aspect of the tongue, thickened lips with nodules, thickened upper eyelids, broadened nasal bridge, thickened corneal nerves and dilated, symmetrical, pedunculated nodules on the cheek mucosa. The patient described has most of these characteristics. Radiographic features of the jaws which have not been previously described are reported. These include a markedly enlarged and bifurcated inferior alveolar canal and shortened roots of the lower incisor teeth. Due to the lethality of the disease, patients who present with the above physical characteristics must be further investigated to exclude MEN2b.

Adrenal Gland Neoplasms

Aneurysms of the visceral arteries.

Visceral artery aneurysms are uncommon. During the period 1975-88, 32 patients were treated at the Royal Melbourne Hospital for true and false visceral artery aneurysms. There were 18 males and 14 females, with an age range of 12-86 years. Of the 32 patients, 26 were symptomatic and six were asymptomatic. True aneurysms were found in 20 patients and false aneurysms in 10. A further two were dissecting aneurysms. Of the arteries involved, 17 were renal, six were hepatic, five were splenic, one was superior mesenteric, one was left colic and there were two patients with aneurysms at multiple sites. Aetiological factors included atherosclerosis, fibromuscular dysplasia, pancreatitis, and trauma. Only one patient presented in pregnancy. Rupture occurred in 12 patients and two died as a result of this complication. All the true hepatic artery aneurysms presented in this way. Pre-operative investigations included plain radiology, computerized tomography with contrast, nuclear scanning and selective angiography. Operative treatment was required in 22 cases, 12 as an emergency and 10 as an elective procedure. Surgical options included aneurysm excision with or without arterial reconstruction, aneurysmorrhaphy with flap arterioplasty, or ligation of the aneurysm. Embolization was successfully employed in two patients and eight were merely observed without complications. Surgical therapy is recommended for any patient with symptoms, for any woman of child-bearing age and for all hepatic artery aneurysms.

Adolescent

Biliary obstruction due to intraductal tumor.

Five unusual cases of bile-duct obstruction are presented. There were three cases of intrabiliary hepatoma, one of intrabiliary colon cancer metastasis, and one cystadenoma of the bile-duct. The importance of an accurate diagnosis is emphasized, as these tumours can be treated by surgery or by biliary stenting, sometimes with prolonged survival.

Aged

Extracorporeal shock-wave lithotripsy and the management of common bile-duct calculi.

Endoscopic sphincterotomy is the treatment of first choice for stones that remain in the bile duct after cholecystectomy. There is a small group of patients in whom this technique is not successful; many of these patients carry a high risk for surgery because of their age or associated medical conditions. A variety of non-surgical techniques is available; however, none is well established. We have used an in-vitro model to show that human gallstones are fragmented readily by shock-wave lithotripsy. Two elderly frail patients with difficult bile-duct stones have been treated successfully by extracorporeal shock-wave lithotripsy. The bile ducts were cleared of stones and the patients suffered no adverse effects. Extracorporeal shock-wave lithotripsy is a new and promising alternative to the current non-surgical techniques for the management of bile-duct stones.

Aged

Malignant masquerade at the hilum of the liver.

Eight patients with biliary obstruction and a pre-operative diagnosis of a neoplastic lesion at the confluence of the hepatic ducts were found postoperatively to have benign disease. Cholangiography was highly suggestive of a malignant stricture in all patients. Angiography performed in six patients indicated that the lesions were potentially resectable. Seven patients underwent elective surgery; in six the ductal confluence including the lesion was removed, without mortality. Six patients are alive, five of them totally asymptomatic, in a median follow-up of 32 months. Many patients with hilar strictures are treated for what appears radiologically to be a neoplastic lesion. Since treatment often involves the placement of an endoprosthesis or palliative surgery, without histological diagnosis, some of these patients with benign disease are likely to be treated inappropriately, unless they are considered for a curative resection.

Adult

Surgical approaches in primary sclerosing cholangitis.

Primary sclerosing cholangitis is an uncommon condition, generally considered to carry a poor prognosis. Based on the distribution of the biliary strictures, treatment groups can be defined. Those patients with localized hilar or predominantly extrahepatic strictures are most likely to be suitable for biliary-enteric bypass and the use of surgical techniques developed for the management of high bile duct strictures are allowing improvement in the results of surgery in this form of the disease. After 3 months to 3 years follow-up (median 16 months) nine of 12 patients treated by surgical biliary decompression are asymptomatic.

Adolescent