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Biomedical subjects

H R van Buuren

Publications and source records attributed to H R van Buuren.

At least 19 recordsLinked to original sources

[Hepatic hydrothorax].

Hepatic hydrothorax was diagnosed in four patients with liver cirrhosis, three men aged 65, 41, and 48 and a woman aged 48. They presented with either right-sided or bilateral pleural-fluid accumulations in the absence of cardiopulmonary disease. In the first man with no concurrent ascites, the disorder was missed, resulting in prolonged chest tube drainage, multiple severe complications and death. In the 41-year-old man chest tube drainage was also associated with complications including renal failure and encephalopathy. Pleurodesis was effective in the woman while in the remaining man hepatic hydrothorax was only a temporary, asymptomatic finding. Pleural effusions in cirrhotic patients should be considered and managed as hepatic hydrothorax unless diagnostic studies reveal a different aetiology. Absence of ascites is not uncommon and should not delay the correct diagnosis. The gradient between pleural and serum albumin concentration is typically more than 11 g/l. Prolonged chest tube drainage is dangerous and should be avoided. In cases refractory to salt restriction and diuretic therapy, transjugular introduction of an intrahepatic portosystemic shunt is the treatment of choice. Recently, pleurodesis combined with thoracoscopic repair ofdiaphragmatic defects has been reported as a potentially effective form of therapy.

Adult↗

[A retrospective study on the role of antibodies against soluble liver antigen (anti-SLA antibodies) and other autoantibodies in the diagnostics of autoimmune hepatitis].

OBJECTIVE: To determine the diagnostic value of antibodies against soluble liver antigen (anti-SLA antibodies) and a number of other antibodies for the diagnosis ofautoimmune hepatitis (AIH). DESIGN: Retrospective. METHOD: Anti-SLA, antinuclear antibodies (ANA), antibodies against smooth muscle (anti-SMA), anti-neutrophil cytoplasm antibodies (peri-nuclear pattern; pANCA) and antibodies against liver-kidney microsomal antigen type 1 (anti-LKM-1) were determined in the sera of 97 patients with AIH and 121 patients with other liver disorders including viral, drug-related and alcoholic liver disease. The sensitivity and specificity of each of the antibodies, or a combination ofantibodies, were calculated for the diagnosis 'AIH'. RESULTS: Anti-SLA antibodies were found only in AIH patients (specificity: 100%); 1 in 7 AIH patients (14%) had these antibodies and in 2% they were the only detectable antibodies. Anti-LKM-1 antibodies also showed a 100% specificity for AIH although the sensitivity was much lower (2%). Whilst the sensitivity of ANA (53%), pANCA (39%) and SMA (39%) was higher, the specificity of these antibodies for AIH was lower. 20% of AIH patients tested negative for all autoantibodies. The simultaneous presence of more than one antibody increased the probability of AIH diagnosis. CONCLUSION: When AIH is suspected, the presence of one or a combination ofanti-SLA, ANA, anti-SMA, anti-LKM-1 and pANCA antibodies is helpful for the often difficult differential diagnosis between AIH and other liver disorders. Anti-SLA antibodies are specific for AIH and appear to be a useful diagnostic parameter.

Autoantibodies↗

[Fever as a sign of inflammatory syndrome in a female patient with hepatic haemangioma].

A 41-year-old patient presented with fever, night sweats, general malaise, abdominal pain, and substantial weight loss. Laboratory analysis suggested an inflammatory process. Diagnostic imaging revealed a hepatic haemangioma with a diameter of 20 cm. Because such giant haemangiomas of the liver can lead to inflammatory syndrome, the tumour was surgically removed. Pathological analysis confirmed the clinical diagnosis and evidence of extensive thrombosis and other vascular defects was found. Following treatment, the symptoms resolved without further complications. In patients with a giant haemangioma in the liver who present with an inflammatory syndrome, the haemangioma should be considered as the causal factor. For these patients, resection is the treatment of choice.

Abdominal Pain↗

A pilot study exploring the role of glucocorticoid receptor variants in primary biliary cirrhosis and primary sclerosing cholangitis.

BACKGROUND: In primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) significant therapeutic effects of glucocorticoids have not been documented. The most important clinical problem in patients with these diseases is fatigue, which is occasionally invalidating. Abnormalities in the hypothalamo-pituitary-adrenal axis have been suggested as a cause of fatigue. Most effects of glucocorticoids are mediated by the glucocorticoid receptor (hGR alpha). Recently a causative role for a splicing variant of the glucocorticoid receptor (hGR beta) has been proposed in glucocorticoid resistance in asthma and ulcerative colitis, whereas another splicing variant (hGR P) might be associated with glucocorticoid-resistant haematological malignancies. The aims of the present pilot study were to assess abnormalities in glucocorticoid receptor expression and to relate these abnormalities to the development of fatigue and to disease activity and severity in autoimmune cholestatic liver disease. METHODS: Five fatigued and five nonfatigued patients with PBC or PSC were included, and the results were compared with healthy controls. RESULTS: The expression of hGR P was not different from controls, but hGR beta mRNA was significantly increased (p=0.02) and hGR alpha mRNA decreased (p=0.015). There were no significant differences between fatigued and nonfatigued patients. A significant negative correlation between the serum activity of alkaline phosphatase and hGR alpha and hGR P mRNA was found. CONCLUSION: Although there was no relation with fatigue, abnormalities in hGR expression appear to occur in patients with these diseases, and may play a role in its pathophysiology and the poor response to glucocorticoid treatment.

Adult↗

A randomised study on the efficacy and safety of an automated Tru-Cut needle for percutaneous liver biopsy.

BACKGROUND: We studied whether the theoretical advantages of a spring-loaded liver biopsy needle exist in clinical practice and if so if they are dependent upon the experience of the physician performing the biopsy. METHODS: In a stratified randomised study we enrolled 215 consecutive patients to compare the safety and efficacy of a new automatic biopsy gun (Acecut) with that of a standard Tru-Cut needle. RESULTS: A total of 464 biopsies were performed. The endpoints of the study were number of needle passes needed per patient, tissue yield of each needle pass and post-biopsy complications. The performance of the automatic needle was superior and more consistent with respect to tissue yield compared with the Tru-Cut needle (median yield 100% and 80%, respectively; p < 0.001). The difference was most marked for inexperienced physicians. There was no difference between the two needles in the number of passes needed. More post-biopsy pain and post-biopsy use of analgesics were observed in the automatic needle group (p = 0.04). CONCLUSION: The automatic Tru-Cut needle offers an advantage, particularly for physicians with no or limited experience in liver biopsies. However more post-biopsy pain and post-biopsy use of analgesics were observed in the automatic needle group.

Adult↗

[Intrahepatic cholestasis of pregnancy].

Intrahepatic cholestasis of pregnancy occurs mainly in the final months of pregnancy, and is characterised by pruritus. Foetal morbidity and mortality are increased. The disorder is probably caused by a genetic defect in hormonal metabolism which becomes manifest during the altered hormonal balance in pregnancy. The total serum bile acid concentration is the diagnostic hallmark of the disease. Other routine laboratory tests have a low sensitivity and specificity. An active obstetric management with routine foetal assessments, caesarean section if the foetal condition worsens, and termination of the pregnancy at 37-38 weeks, probably prevents a large proportion of complications. Ursodeoxycholic acid treatment should be considered especially in patients with severe pruritus or complications in previous pregnancies.

Adult↗

Transjugular intrahepatic portosystemic shunt (TIPS): indications and long-term patency.

BACKGROUND: Since the introduction of TIPS (the transjugular intrahepatic portosystemic shunt) into clinical practice in 1989, substantial knowledge, partially derived from controlled trials, has become available regarding technical and clinical aspects of the procedure. A number of prospective studies have assessed the long-term patency of radiological shunts, the recognized main technical weakness of the procedure. METHODS: Review of published data regarding the optimal indications and long-term patency of TIPS. RESULTS: Information on the long-term patency of TIPS is surprisingly scarce. Within 2 years of TIPS creation, re-interventions to re-establish or maintain the patency of the shunt are required in 70%-90% of patients, and in 20%-40% total occlusion develops. Limited available data suggest, however, that in about 80% of patients the shunt is patent after 3-5 years. There is consensus that TIPS is a main, second-line treatment option for variceal haemorrhage not responding to other therapies. Although widely used for treating refractory ascites, gastric variceal bleeding and Budd-Chiari syndrome, these indications require more study. A number of other potential indications remain poorly defined. CONCLUSION: TIPS is a major treatment modality to manage the complications of portal hypertension and Budd-Chiari syndrome. The available data indicate that TIPS is not only a short-term treatment option but may provide long-term portosystemic decompression. Technical improvements, e.g. the use of covered or drug-eluting stents, are essential to reduce the high rate of shunt dysfunction.

Budd-Chiari Syndrome↗

Anthranoid self-medication causing rapid development of melanosis coli.

It is widely known that long-term use of anthranoid-containing laxatives is the cause of melanosis coli. We describe a case of melanosis coli, which occurred in a 39-year-old liver transplant patient who took an over-the-counter product containing aloe, rheum and frangula. The typical brownish pigmentation of the colonic mucosa developed in a period of ten months. The anthranoid medication was stopped and follow-up colonoscopy one year later showed normal looking mucosa once more. However, in contrast to previous examinations, a sessile polypoid lesion was found in the transverse colon. Histology showed tubulovillous adenoma with extensive low-grade dysplasia. Since there have been preliminary reports suggesting a possible role of anthranoid-containing laxatives in the development of colorectal adenomas and cancer, their use should be discouraged.

Adult↗

No beneficial effects of transdermal nicotine in patients with primary sclerosing cholangitis: results of a randomized double-blind placebo-controlled cross-over study.

BACKGROUND/AIMS: Smoking is associated with a decreased risk of primary sclerosing cholangitis. We aimed to explore the therapeutic efficacy of and tolerance for transdermal nicotine treatment in this disease. METHODS: Twelve patients (11 males; 37 +/- 6 years; six with ulcerative colitis) who did not achieve complete biochemical remission on ursodeoxycholic acid (14 mg/ kg/day) were treated in a randomized cross-over trial with transdermal nicotine (15 mg/day) or a placebo, each for 8 weeks (4-week washout period between treatments). RESULTS: One patient developed de novo ulcerative colitis and two did not complete the entire protocol because of intercurrent bacterial cholangitis. Baseline values [mean (range)] were: bilirubin, 1.3 (0.5-2.6); alkaline phosphatase (APh), 2.5 (1.4-4.7); gamma-glutamyl transpeptidase (gammaGT), 7.7 (0.7-38); aspartate aminotransferase (AST), 1.9 (0.5-3.2); alanine aminotransferase (ALT), 2.4 (0.4-7.3); and bile salts, 10.9 (2.1 -39) times the upper limit of normal. No significant effect on pruritus or fatigue was noted during either period, but a small increase in bodyweight was observed during placebo treatment. No significant differences were observed between the two treatment modalities after 8 weeks in bilirubin (nicotine versus placebo, +13% versus -6% change from baseline), APh (-3% versus -17%), gammaGT (-11% versus -13%), AST (+2% versus -10%), ALT (-1% versus -11%) or bile salts (+36% versus -3%). CONCLUSION: Transdermal nicotine does not seem to have a clear short-term beneficial effect in primary sclerosing cholangitis treated with ursodeoxycholic acid.

Administration, Cutaneous↗

Jaundice in non-cirrhotic primary biliary cirrhosis: the premature ductopenic variant.

The clinical and pathological findings of four females with primary biliary cirrhosis (PBC) with an unusual and hitherto not well recognised course are reported. Patients suffered severe pruritus and weight loss with progressive icteric cholestasis which did not respond to such treatments as ursodeoxycholic acid and immunosuppressives. In all cases liver histology revealed marked bile duct loss without however significant fibrosis or cirrhosis. Further diagnostic studies and repeat biopsies confirmed the absence of liver cirrhosis as well as other potential causes of hyperbilirubinaemia. Comparison of the fibrosis-ductopenia relationship for our cases with that for a group of 101 non-cirrhotic PBC patients indicated that in the former the severity of bile duct loss relative to the amount of fibrosis was significantly higher. The proportion of portal triads containing an interlobular bile duct was 3%, 4%, 6%, and 10% compared with 45% (median; range 8.3--100%) for controls (p<0.001). Three patients received a liver transplant 6--7 years after the first manifestation of PBC because of progressive cholestasis, refractory pruritus, and weight loss, while the fourth patient is considering this option. In one case cirrhosis had developed at the time of transplantation while the others still had non-cirrhotic disease. These cases suggest that cholestatic jaundice in non-cirrhotic PBC may be secondary to extensive "premature" or accelerated intrahepatic bile duct loss. Although the extent of fibrosis may be limited initially, progression to cirrhosis appears to be inevitable in the long run. Despite intact protein synthesis and absence of cirrhotic complications, liver transplantation in the pre-cirrhotic stage for preventing malnutrition and to improve quality of life should be considered for these patients.

Adult↗

High prevalence of autoimmune hepatitis among patients with primary sclerosing cholangitis.

BACKGROUND/AIMS: Traditionally, autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are regarded as separate disease entities. We report on a group of patients that suggests the existence of an overlap syndrome of the two conditions and on the prevalence of this syndrome among patients with PSC. Furthermore, the impact of the recently revised AIH scoring system for diagnosing AIH in this context was assessed. METHODS: Retrospective analysis of consecutive patients of a tertiary referral centre for liver disease with a diagnosis of PSC. RESULTS: Diagnosis of the overlap syndrome was established for nine patients (8%) of a total group of 113 PSC patients. Four patients initially presented with features of AIH and in five cases PSC was diagnosed first. All patients responded to immunosuppressive therapy; in three cases long-term remission was achieved. Three patients underwent liver transplantation after 4 months and 7 and 9 years, respectively. The original and revised versions of the AIH scoring system gave essentially the same results in the patients with the PSC-AIH overlap syndrome. CONCLUSIONS: Patients with overlapping features of AIH and PSC may be more common than is currently assumed. Recognition of this syndrome is of clinical significance, considering the important therapeutical consequences.

Adolescent↗

Management of osteoporosis in primary biliary cirrhosis.

Osteoporosis is not a significant problem in the majority of patients with primary biliary cirrhosis (PBC). However, substantial bone-related morbidity may occur in patients with advanced disease, in particular after liver transplantation. The cause of osteoporosis in PBC is multifactorial, and pathophysiological mechanisms specifically related to PBC have not been defined. In general, the principles of management followed in post-menopausal osteoporosis also apply in chronic liver disease. Dual energy X-ray absorptiometry is currently the method of choice for monitoring bone mineral density. Avoidance of conditions with potential negative effects on bone mass, and maintaining adequate serum vitamin D levels and calcium intake form the cornerstone in preventing osteoporosis. Bisphosphonates are the most logical choice when specific medical treatment of PBC-associated osteoporosis is indicated, as well as for preventing bone loss during glucocorticoid treatment and after liver transplantation. Recent studies suggest that active vitamin D analogues are effective alternatives in the post-transplant setting.

Absorptiometry, Photon↗

Prospective risk assessment of endoscopic retrograde cholangiography in patients with primary sclerosing cholangitis. Dutch PSC Study Group.

BACKGROUND AND STUDY AIMS: Direct endoscopic retrograde cholangiopancreatography (ERCP) has become the standard for establishing the diagnosis of primary sclerosing cholangitis (PSC), while endoscopic procedures play an increasingly important therapeutic role. However, many believe that this procedure carries a significant risk of infection and other complications. We assessed the incidence of complications within 1 week of ERCP in patients with PSC. PATIENTS AND METHODS: In a multicenter study, patients who underwent ERCP for (suspected) PSC were prospectively followed for the occurrence of complications after the procedure. RESULTS: A total of 106 ERCPs performed in 83 patients were evaluated. Complications occurred on ten occasions (9%): pancreatitis (n = 3), cholangitis (n = 2), increase of cholestasis (n = 2), postsphincterotomy bleeding (n = 1), cystic duct perforation (n = 1), and venous thrombosis (n = 1). All complications resolved quickly with proper therapy. Complications were more likely when ERCP was done to evaluate specific complaints such as jaundice or recurrent cholangitis (9/59) than after a purely diagnostic ERCP (1/47 relative risk [RR] 7.2, 95% confidence interval [CI] 1.00 to 153). Therapeutic interventions performed during ERCP (e.g. placement of endoprosthesis, dilation of strictures) also increased the risk of postprocedural complications (RR 4.5, 95 % CI 0.94 to 30). CONCLUSIONS: ERCP is a safe method for establishing the diagnosis of PSC in asymptomatic patients (2 % complication rate). Although ERCP in symptomatic patients carries a higher risk (14%), this can be justified by the benefits of endoscopic therapy.

Adolescent↗

Budesonide or prednisone in combination with ursodeoxycholic acid in primary sclerosing cholangitis: a randomized double-blind pilot study. Belgian-Dutch PSC Study Group.

OBJECTIVE: PSC has characteristics of an (auto)immune-mediated disease: however, few studies have evaluated corticosteroid therapy for this disorder. METHODS: We performed an 8-wk double-blind randomized pilot study to assess the effects of additional treatment with 9 mg budesonide (n = 6) versus 3 mg budesonide (n = 6) versus 10 mg prednisone (n = 6) in patients who had been treated with UDCA (mean dose, 12 mg/kg/day) for at least 5 months without achieving biochemical remission. Pruritus and fatigue were evaluated using visual analog scales. Serum liver biochemistry was measured every 4 wk. At entry and at the end of the trial, adrenocorticotrophic hormone (ACTH) and dehydroepiandrosterone (DHEA) were measured to assess effects on the pituitary-adrenal axis. Duodenal bile was collected for assessment of biliary corticosteroid activity. RESULTS: Pruritus decreased significantly more in the prednisone group compared to both the 3-mg and the 9-mg budesonide groups (p < 0.05). Alkaline phosphatase (mean: -23.4%; p = 0.03) and IgG (mean: -16.2%; p = 0.04) decreased in the prednisone group, whereas bilirubin, gamma-glutamyl transferase, aspartate aminotransferase, and alanine aminotransferase did not change significantly. No significant clinical or liver biochemical changes were observed in the 3-mg and 9-mg budesonide groups. Significantly larger drops in serum ACTH were found in the 10-mg prednisone group (-40.7%; p = 0.04) and 9-mg budesonide group (-36.6%; p = 0.02) compared to the 3-mg budesonide group (+ 19.0%). No significant differences in percentage change in baseline values for DHEA between the three treatment arms were found. Mononuclear cell proliferation assays did not demonstrate corticosteroid activity in bile. Autoimmune hepatitis was observed in one case (9 mg budesonide) when corticosteroids were tapered off. CONCLUSION: The results of this pilot study suggest only minor beneficial short-term effects of prednisone but not budesonide on symptoms and serum liver tests in UDCA-treated PSC patients.

Adrenal Cortex Hormones↗