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Biomedical subjects

D M Painter

Publications and source records attributed to D M Painter.

At least 19 recordsLinked to original sources

Fraternal sisters with adult polycystic kidney disease and adenoma of the ampulla of Vater.

The cases of two fraternal sisters with symptomatic biliary obstruction due to adenomas of the ampulla of Vater are reported. Both sisters had autosomal dominant adult polycystic kidney disease. There are no previous reports of a familial occurrence of ampullary adenomas in the absence of familial adenomatous polyposis, nor has an association between autosomal dominant polycystic kidney disease and ampullary adenoma been described. The coexistence of both disorders in these sisters raises the possibility of a genetic link between autosomal dominant polycystic kidney disease and ampullary adenoma.

Adenoma

Histopathological prediction of hepatitis B recurrence in liver allografts.

The aim of this study was to review the clinical, serological and histopathological features of 20 patients who underwent orthotopic liver transplantation for hepatitis B virus (HBV) related liver disease in an attempt to identify factors which predict recurrence of HBV infection, pattern of disease and disease severity. Hepatitis B recurrence occurred in 11 patients (55%). Positive immunoperoxidase staining for HBsAg and HBcAg of the hepatectomy specimen were shown to have a sensitivity (82% and 64% respectively) and specificity (55% and 66%) in predicting recurrence. This was comparable to serological markers such as HBV DNA (36% sensitivity and 89% specificity). The staining for HBcAg in some cases was weak, focal and had a reticular pattern. Therefore, examination of multiple blocks with a high magnification was necessary to detect HBcAg staining. Negative staining of multiple sections for HBsAg and HBcAg were shown to have a good correlation with clearing of HBV infection (5 out of 6 patients). If recurrence occurred, the range of histological and clinical patterns of HBV recurrence was varied, with severe disease occurring in 5 patients. Neither pre-transplant serology nor hepatectomy immunoperoxidase staining characteristics were shown to be of value in predicting the pattern or the severity of disease once HBV recurrence had occurred. This study indicates that lack of staining for HBV antigens on multiple sections of the native liver may predict a subgroup of HBsAg positive transplant patients who will not suffer from recurrent disease. Once disease did occur however, markers for severity of recurrence were not identified.

Base Sequence

Hepatic infarction and nodular regenerative hyperplasia of the liver with associated anticardiolipin antibodies in a young woman.

An 18-year-old woman developed acute congestive hepatic infarction in the presence of a circulating anticardiolipin antibody. She subsequently developed nodular regenerative hyperplasia of the liver (NRHL) with associated portal hypertension. Unlike previously reported cases of NRHL, this case offers a prospective follow-up of the development of NRHL. Its occurrence following an episode of hepatic venous impairment in an otherwise normal liver supports the hypothesis that NRHL may develop as a consequence of diminished hepatic venous drainage.

Adult

Clinical assessment and incidence of hepatitis C RNA in 50 consecutive RIBA-positive volunteer blood donors.

OBJECTIVES: (i) To assess evidence of liver disease in 50 consecutive volunteer blood donors who were anti-hepatitis C virus (anti-HCV) antibody positive and who were referred to one hepatologist; (ii) to assay for viral RNA in serum in these patients. SETTING: Royal Prince Alfred Hospital, a teaching hospital of the University of Sydney. PATIENTS: Fifty people who were detected by the NSW Red Cross Blood Transfusion Service to be anti-HCV antibody positive and to have a positive result on recombinant immunoblot assay (RIBA) were assessed by one hepatologist for symptoms, signs and biochemical evidence of hepatic dysfunction. These patients were consecutive referrals from this source. Sixteen of these patients also consented to liver biopsy assessment. All patients had serum assayed for viral RNA by polymerase chain reaction with a combination of 3' and 5' primers. RESULTS: The 50 blood donors consisted of 28 men and 22 women, with a mean age of 34.5 years. Forty-six patients were asymptomatic. Only six had a past history of hepatitis while 14 had minor signs of chronic liver disease. In 28, injecting drug use was thought the most likely source of exposure to HCV. The minimal mean time since exposure to HCV in these patients was 8.8 +/- 5.2 years. Eight patients had received a blood transfusion at a mean time of 15.0 +/- 9.8 years from the time of consultation. The mean maximum level of alanine aminotransferase (ALT) in all 50 patients was 102.8 U/L. Five patients had persistently normal ALT levels; another 22 had at least one normal ALT level. Liver biopsies indicated chronic persistent hepatitis in 11 patients, mild chronic active hepatitis in three patients and more severe chronic active hepatitis in one. One patient had cirrhosis on biopsy. Forty-two patients had viral RNA detected in serum. CONCLUSION: Chronic infection with HCV in blood donors was invariably asymptomatic; 78% of patients had no signs of chronic liver disease and 68% had a maximum hepatic transaminase level of less than 100 U/L. Although severe liver disease was seen in two of 16 biopsies, the majority of these patients have mild liver disease despite a mean of about 10 years since exposure to the virus. Eighty-four per cent of patients had evidence of viral RNA in serum.

Adult

Sulfasalazine-induced fulminant hepatic failure.

We report two cases of massive hepatic necrosis associated with sulfasalazine. Both patients had underlying inflammatory bowel disease. One of the patients had a history of an ill-defined autoimmune disorder (Sjögren's syndrome). Symptoms and signs of a generalized hypersensitivity reaction were present in both patients. One patient died of a subarachnoid hemorrhage while awaiting transplant, the second died 2 weeks after transplant from disseminated aspergillosis. These two cases remind us of one of the potential hazards of sulfasalazine at a time when alternative therapies are now available.

Adult

Inflammatory pseudotumor of the liver causing biliary obstruction. Treatment by biliary stenting with 5-year follow-up.

Inflammatory pseudotumors of the liver are rare lesions that are becoming increasingly recognized, possibly because of frequent use of abdominal computed tomography (CT) and ultrasonography. Most previously reported cases have been treated by hepatic resection. For this reason, we describe a patient with obstructive jaundice secondary to an inflammatory hepatic pseudotumor in the porta hepatis. Bilateral internal biliary stents were inserted, and the patient remains well 5 years later. The clinicopathologic features of inflammatory pseudotumors of the liver are described, as are the implications of accurate diagnosis. There have been no previous reports of biliary stenting in management of inflammatory hepatic pseudotumors.

Adult

A case of AIDS presenting as intra-oral malignant lymphoma.

A range of oral manifestations in a patient who was subsequently confirmed as infected with the human immune deficiency virus are presented. Most of these lesions have been previously reported in the literature, and represent opportunistic infection changes resulting from immune deficiency. The rare occurrence of a malignant large cell lymphoma intra-orally in this patient is also reported. The need for clinicians to treat all oral lesions as potentially infective and to wear gloves routinely is stressed.

Acquired Immunodeficiency Syndrome

A quantitative analysis of T lymphocyte populations in human liver allografts undergoing rejection: the use of monoclonal antibodies and double immunolabeling.

The aim of this study was to quantitate T-cell populations infiltrating portal tracts, bile ducts and hepatic lobules in 82 biopsy specimens from 25 patients after orthotopic liver transplantation. Biopsy specimens taken immediately after revascularization of the graft were used as controls. Patients studied include 18 with initial rejection episodes, 11 with unresolved rejection, five with vanishing bile duct syndrome and eight patients with other forms of liver injury. Quantitation was done in a blinded fashion for the first 20 biopsy specimens. A double immunolabeling technique was used to simultaneously immunolabel bile duct structures (with anti-major histocompatibility complex class II or antikeratins) and lymphoid populations (with anti-CD2, anti-CD4 or anti-CD8). This facilitated the accurate quantitation of intraepithelial lymphocytes within bile ducts. This technique also enabled simultaneous detection of CD4 and CD8 antigens on lymphocytes in portal tracts. The predominant lymphocyte subtype within biliary epithelium during acute and chronic rejection was of the CD2+/CD8+ phenotype. CD8+/CD4+ ratio in bile ducts was approximately 5:1 in acute, unresolved and chronic rejection. In vanishing bile duct syndrome, double immunolabeling enabled the detection of destroyed interlobular bile duct remnants that were not apparent on routine hematoxylin and eosin staining. Attached to some of these structures were CD8+ lymphocytes. Lobular CD8+ cells were not prominent in acute rejection but increased significantly in biopsy specimens from patients with unresolved and chronic rejection. In chronic rejection, a selective increase was seen in these CD8+ cells in centrizonal regions.(ABSTRACT TRUNCATED AT 250 WORDS)

Antibodies, Monoclonal

Widespread cutaneous necrosis associated with cardiolipin antibodies.

Many clinical phenomena have been described in association with antiphospholipid antibodies, which are measured by a variety of techniques. The relationship of each assay is not completely clear, nor is it apparent which assay correlates best with the various clinical conditions. We describe a patient with anticardiolipin antibodies but without lupus inhibitor who had widespread cutaneous necrosis and underlying thrombosis in dermal blood vessels.

Adult

Nodular regenerative hyperplasia mimicking cirrhosis of the liver.

Nodular regenerative hyperplasia of the liver usually presents with signs of portal hypertension with little evidence of obvious liver disease. We report a 47 year old man who presented with clinical signs of decompensated cirrhosis, recurrent encephalopathy, and tense ascites but at liver transplant was found to have nodular regenerative hyperplasia associated with a portal vein thrombosis.

Diagnosis, Differential

Liver transplantation: an Australian experience.

During a 4 and a half-year experience, 283 patients were referred to the Australian National Liver Transplant Unit. Sixty (21%) were children. The major causes of liver failure in the adults were chronic active hepatitis (27%), primary biliary cirrhosis (13%), primary sclerosing cholangitis (12%), fulminant hepatic failure (9%), alcoholic cirrhosis (9%), and malignancy (9%). In the children they were biliary atresia (43%) and inborn errors of metabolism (18%). One hundred and ninety-seven (69%) were accepted for liver transplantation. Of 111 (39%) accepted for early transplantation, 18 (16%) died before a donor became available. There were 319 possible organ donors. Ninety patients (24 of them children) received 100 grafts. Sixty-three (70%) patients survived. For all recipients, 1-year survival was 73%. Two-, 3- and 4-year survivals were 67%. One- to 4-year survivals for adults with benign conditions were 76%, contrasting with results for those with hepatic malignancy (20% 1-year survival). Children weighing greater than 8 kg did well whether they received whole grafts (80% 1- to 4-year survival) or reduced grafts (75% 1- to 4-year survival). Infants weighing less than 8 kg who received reduced adult grafts did significantly worse (20% 1- to 4-year survival). All 8 (100%) patients with fulminant hepatic failure who received grafts survived, including 3 who received ABO-incompatible grafts, though 2 of these subsequently required retransplantation. Rehabilitation of survivors was excellent with 91% of adults and 94% of children pursuing normal activities. Only 2 (2%) grafts failed with primary nonfunction, both in infants because of infarction. Graft survival was significantly worse (p less than 0.01) in patients with a positive direct crossmatch test against the donor. We calculate that the need for liver transplantation in Australia is approximately 7 per million of population per year. Increased donor offers are required to avoid deaths of patients on the waiting list. Reduced-size adult livers are successful for children and have alleviated considerably the critical shortage of pediatric donor livers. Liver transplantation is a highly satisfactory treatment for patients with benign liver disorders but not for those with malignant conditions. Patients with fulminant hepatic failure not responding to conservative therapy should be treated by liver transplantation. In this and other urgent circumstances, an ABO-incompatible liver may be lifesaving though retransplantation with an ABO-compatible liver may subsequently be required. The results of liver transplantation for nonmalignant conditions have improved steadily with clinical experience, with 1- and 2-year patient survivals during the past 28 months of 85%.

Adult

Decision-making in 100 patients referred to the Australian National Liver Transplantation Unit.

One hundred patients were referred to the Australian National Liver Transplantation Unit between January 1986 and August 1987. The commonest disorders for referral were chronic active hepatitis in adults (22 cases), fulminant hepatic failure (14), primary biliary cirrhosis (PBC) (12) and primary sclerosing cholangitis (PSC) (10). Of the 100 patients 31 were activated for transplantation while 35 were deferred and 34 were found to be unsuitable. The decision-making in these patients is discussed according to disease category. Timing of transplantation in PBC and PSC seemed clearcut, with 50% of referred patients being activated. However, major difficulties in timing of transplantation were found, particularly in patients with chronic active hepatitis (CAH) and fulminant hepatic failure (FHF). Of 36 patients with CAH (22) or FHF (14) only five were put on the active waiting list and only two were transplanted. Overall, 25 of the 31 patients underwent orthotopic liver transplantation, with 16 alive two-30 months later.

Adult

Danazol and hepatic neoplasia: a case report.

Although hepatic side-effects of danazol are well known, the occurrence of hepatic tumours is not well documented. We report a case of hepatocellular adenoma occurring in a 35-year-old woman with endometriosis who had been taking danazol over a three year period.

Adult

The role of nuclear medicine in liver transplantation.

The role of hepatobiliary isotopic scanning after liver transplantation was assessed in the first 12 adult patients in the Pilot National Liver Transplantation Programme who underwent this procedure at the Royal Prince Alfred Hospital. Iminodiacetic-acid derivative (HIDA) studies were useful in the non-invasive assessment of hepatic function and biliary excretion. The following four clinical situations were assessed by hepatobiliary scans: early graft function; rejection episodes; vascular complications; and biliary-tract problems. Nuclear medicine has an important and valuable role in the postoperative monitoring of patients after liver transplantation. The studies are non-invasive and can be performed readily at the bedside in the intensive care unit.

Adolescent

Synovial osteochondromatosis of the temporomandibular joint. An historical review with presentation of 3 cases.

Loose bodies in joints have long held the fascination of surgeons and their recognition clearly enjoys a most distinguished antiquity (Pare, 1558; Haller, 1764; Barwell, 1876; Halstead, 1895) despite the rarity of their occurrence. This paper presents an historical review of the subject in conjunction with a report of three cases operated upon by one of the authors (JdeBN). The results have been critically reviewed by pathologists with a catholicity of experience in the field. The condition is singularly uncommon in the jaw joint, and must rank with synovial cyst and para-articular chondroma as an unusual cause of a swelling of firstly the temporomandibular joint and secondly the parotid gland. Earlier workers recognized the value of comparative pathology to illustrate the nature of a genus of tumours and it is educative to read the following: "A good physiological type for the loose cartilaginous bodies which infest joints is furnished by the temporomandibular joint of the skate. A recess communicating with this articular cavity usually contains a collection of smooth cartilaginous bodies, in contour and size like melon seeds." Bland-Sutton (1907).

Adult