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E R Dickson

Publications and source records attributed to E R Dickson.

At least 55 records · Page 3Linked to original sources
PubMed1997

Does antimitochondrial antibody status affect response to treatment in patients with primary biliary cirrhosis? Outcomes of ursodeoxycholic acid therapy and liver transplantation.

Approximately 5% to 10% of patients with features otherwise consistent with primary biliary cirrhosis (PBC) lack antimitochondrial antibodies (AMA). Most of these patients have other autoantibodies, a syndrome recently named "autoimmune cholangitis." We report our experience in patients with AMA-negative PBC treated with ursodeoxycholic acid (UDCA) and/or liver transplantation (OLT). The study of response to UDCA was performed as follows. While recruiting patients for a previously reported multicenter trial, we identified 8 patients with AMA-negative PBC. The patients were given UDCA and followed up at regular intervals. The characteristics of AMA-negative patients at presentation were similar to those of AMA-positive patients with PBC. The clinical outcomes and sequential liver biochemistries of UDCA treatment were also comparable with those of AMA-positive patients. The study of outcome of OLT was performed as follows. We identified OLT recipients at the Mayo Clinic who had clinical, radiological, and histological features compatible with PBC. Their pretransplant AMA status was determined, and each AMA-negative patient was paired with 2 AMA-positive patients. Of 85 OLT recipients with a diagnosis of PBC, 6 (7.1%) were AMA negative, including 1 who had undergone UDCA therapy. After a median of 36 months of follow-up, graft and patient survival rates and subsequent histological changes (disease recurrence and steroid-resistant or late rejections) were comparable in AMA-negative and -positive PBC patients. In summary, in our experience of 13 AMA-negative PBC patients (including 9 who met the criteria for a diagnosis of autoimmune cholangitis), treatment with UDCA or OLT resulted in similar outcomes to those found in AMA-positive patients. We conclude that AMA status does not affect the response in PBC patients to treatment with UDCA or OLT.

Adult↗
PubMed1997

Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis.

BACKGROUND & AIMS: Long-term ursodeoxycholic acid (UDCA) therapy slows the progression of primary biliary cirrhosis. This study examined the effect of UDCA therapy on survival free of liver transplantation in a large group of patients. METHODS: Data from three clinical trials were combined in which patients with primary biliary cirrhosis were randomly assigned to receive UDCA (n = 273) or placebo (n = 275). After 2 years, patients from French and Canadian studies received UDCA for up to 2 years. Patients from the American study remained on their assigned treatment for up to 4 years. RESULTS: Survival free of liver transplantation was significantly improved in the patients treated with UDCA compared with the patients originally assigned to placebo (P < 0.001; relative risk, 1.9; 95% confidence interval, 1.3-2.8). Subgroup analyses showed that survival free of liver transplantation was significantly improved in medium- and high-risk groups (serum bilirubin level, 1.4 to 3.5 or > 3.5 mg/dL; P < 0.0001 and P < 0.03, respectively) and histological stage IV subgroup (P < 0.01). CONCLUSIONS: Long-term UDCA therapy improves survival free of liver transplantation in patients with moderate or severe disease. An effect in patients with mild disease is probably not found because they do not progress to end-stage disease in 4 years.

Adult↗
PubMed1997

The role of prognostic models in the timing of liver transplantation. Application in cholestatic liver diseases.

Prognostic models have been developed for patients with primary biliary cirrhosis and primary sclerosing cholangitis to predict survival without transplantation. In patients undergoing liver transplantation, these models have been used in assessing postoperative mortality and morbidity. Recent data suggest that preoperative recipient physiology, such as impaired functional status or renal insufficiency, is the most important determinant of transplant outcome. Survival, quality of life, morbidities and resource use are the key variables to be considered in the timing of transplantation.

Cholangitis, Sclerosing↗
PubMed1997

Survival algorithms and outcome analysis in primary biliary cirrhosis.

The natural history of primary biliary cirrhosis (PBC) is one of slowly progressive cholestasis with liver damage, development of cirrhosis with its concomitant complications, and death unless the patient undergoes liver transplantation. Natural history studies have identified several variables associated with a decreased survival in patients with PBC. The course of the disease can be divided into three time periods: (1) a presymptomatic phase, probably lasting up to 20 years; (2) a symptomatic phase, with anicteric or mild jaundice, lasting up to 5 to 10 years; and (3) a preterminal or accelerated phase with marked jaundice, lasting up to 2 years. Since the course of the disease is one of slow progression leading to liver failure and death unless liver transplantation intervenes, several investigators have developed statistical models to predict survival. The ability to predict survival for individual patients with PBC has been valuable in the management of these patients, particularly in patient selection and timing of liver transplantation. In addition, survival estimates can be utilized in education and counseling patients and their families. These models may also be used to evaluate the efficacy of new treatments by comparing natural history survival with the survival achieved by therapeutic effect. Over the past several decades, the natural history models of PBC have been developed in the absence of effective medical therapy. The efficacy of liver transplantation and survival following liver transplantation has now been quantitatively established. Future efforts should be aimed at determining not only survival of patients with primary biliary cirrhosis in the presence of effective medical therapy but also at assessing the quality of life and cost-effectiveness of medical therapy and liver transplantation in the management of patients with primary PBC.

Algorithms↗
PubMed1997

Ursodeoxycholic acid delays the onset of esophageal varices in primary biliary cirrhosis.

OBJECTIVE: To address the effect of ursodeoxycholic acid therapy on development of esophageal varices in patients with primary biliary cirrhosis. MATERIAL AND METHODS: We compared, as part of a prospective treatment trial, the risk of varices developing in patients with primary biliary cirrhosis who received ursodeoxycholic acid (13 to 15 mg/kg daily) versus those who received placebo for up to 4 years. Upper endoscopy was performed every 2 years or as indicated clinically. At the end of the 4-year period, all patients in the placebo group were offered ursodeoxycholic acid therapy. During follow-up, the risk of developing endoscopically confirmed varices was assessed. RESULTS: The 180 patients who entered the ursodeoxycholic acid trial were assessed for the presence or absence of varices by esophagogastroduodenoscopy; 139 patients had no varices, and 41 patients demonstrated varices on initial examination. At 4 years, the risk of newly developing endoscopically confirmed varices was 16% for the ursodeoxycholic acid-treated patients and 58% for the placebo-treated patients (P < 0.001). Thus, the use of ursodeoxycholic acid was associated with a significantly lower risk of developing varices in patients with primary biliary cirrhosis. CONCLUSION: In addition to biochemical improvement, delay in death, and prolongation of time to orthotopic liver transplantation, ursodeoxycholic acid has now been demonstrated to decrease the risk of esophageal varices developing in patients with primary biliary cirrhosis.

Aged↗
PubMed1996

Recurrence of nonalcoholic steatohepatitis following liver transplantation.

Patients with nonalcoholic steatohepatitis (NASH) may develop progressive liver dysfunction necessitating liver transplantation (OLT). We report the incidence of recurrent disease and outcome in patients undergoing OLT for NASH. Patients transplanted for NASH were identified according to pretransplant and explant liver histology. Patients with significant alcohol consumption were excluded. Medical records were reviewed to extract pre- and posttransplant data, including sequential body weight, biochemistry, and graft histology. Of 622 liver explants, eight patients had features consistent with NASH. All patients were female with a median age of 58. Seven patients were diagnosed with NASH preoperatively, including three who had undergone jejunoileal bypass. One patient was diagnosed as cryptogenic cirrhosis. At a median of 15 months following OLT, all of the eight patients were alive with no graft failure. Six patients developed persistent fatty infiltration in their graft, three of whom had accompanying hepatocellular degeneration, consistent with a diagnosis of recurrent NASH. In two patients, transition from mild steatosis to steatohepatitis and early fibrosis was observed over one to two years. The patients who did not develop recurrent steatosis had significant weight loss following transplantation, although the length of follow-up was relatively short. Patients undergoing OLT for NASH may develop recurrent steatosis shortly after transplantation, with possible progression to steatohepatitis and fibrosis. Although longer follow-up is necessary to determine the eventual prognosis related to the recurrent fat and fibrosis in the graft, patients with endstage liver disease due to NASH should be considered good candidates for OLT.

Adult↗
PubMed1996

Time course of histological progression in primary biliary cirrhosis.

Histological staging is used for stratification and assessment of treatment efficacy in therapeutic trials for primary biliary cirrhosis (PBC). Knowledge of the rate of progression of the histological changes would be helpful in the design (duration) and conduct of clinical trials. The histological stages were recorded for liver biopsies performed annually on 222 patients during a randomized, placebo-controlled clinical trial in which therapy with D-penicillamine (DPCA) was shown to be ineffective. These data were analyzed using a Markov model to describe the time course of histological progression in PBC. At study entry, 15 patients were stage I, 56 were stage II, 96 were stage III, and 55 were stage IV. Histological progression was observed after 1 year in 41%, 43%, and 35% of the patients, and after 2 years in 62%, 62%, and 50% of the patients who were stage I, stage II, and stage III at entry, respectively. After 4 years biopsies showed cirrhosis in 31% and 50% of the patients in stage I and stage II at entry, respectively. A minority (20%) of the precirrhotic patients showed histological stability; sustained histological regression was rarely observed (2%). Our data suggest that a majority of patients with PBC will progress histologically within 2 years. The distribution of histological stages over time may be helpful in determining the number of patients and length of time necessary to appreciate a treatment effect on histological progression in clinical trials for PBC.

Biopsy↗
PubMed1996

Epitope mapping and reactivity of autoantibodies to the E2 component of 2-oxoglutarate dehydrogenase complex in primary biliary cirrhosis using recombinant 2-oxoglutarate dehydrogenase complex.

Five different target mitochondrial autoantigens recognized by sera from patients with primary biliary cirrhosis (PBC) have been identified as subunits of the following 2-oxo acid dehydrogenase complexes: the pyruvate dehydrogenase complex (PDC), the branched chain 2-oxo acid dehydrogenase complex (BCOADC), and the 2-oxoglutarate dehydrogenase complex (OGDC). Unlike the E2 subunits of PDC (PDC-E2) and BCOADC (BCOADC-E2), the E2 subunits of OGDC (OGDC-E2) reactivity of PBC sera and the reactive epitope of OGDC-D2 have not hitherto been studied in detail. In this report, we took advantage of a recombinant fusion protein for OGDC-E2 to address these issues. Eighty of 268 (29.9%) PBC patient sera but none of the 45 controls reacted with recombinant OGDC-E2. The recombinant OGDC-E2 was judged to express the immunodominant epitope, because when sera from patients with PBC were preabsorbed with the recombinant fusion protein, such sera were depleted of reactivity against 48 kD OGDC-E2 when probed on beef heart mitochondria (BHM) but retained reactivity toward PDC-E2 and/or BCOADC-E2. Furthermore, affinity-purified PBC sera against recombinant OGDC-E2 reacted only with native OGDC-E2 and not with any other enzyme components of the 2-oxo acid dehydrogenase complex. Antimitochondrial autoantibodies (AMA) against OGDC-E2 included immunoglobulin (Ig)G2, IgG3 and IgM and the relative titers were as follows: IgG2 > IgG3 > IgM. Finally, using overlapping recombinant polypeptides, it was determined that a minimum of 81 amino acids (residues 67-147) corresponding to the lipoyl domain of OGDC-E2 are necessary for reactivity, suggesting that a conformational autoepitope is recognized by AMA. These data suggest that each of the 2-oxo acid dehydrogenase enzymes has distinct antigenicity despite their similarities in structure and function. The availability of recombinant OGDC-E2 autoantigen will allow the design of additional studies to further our understanding of the role of mitochondrial autoantigens in the pathogenesis of PBC.

Animals↗
PubMed1996

Use of a designer triple expression hybrid clone for three different lipoyl domain for the detection of antimitochondrial autoantibodies.

The detection of antimitochondrial autoantibodies (AMAs) is critical in the diagnosis of primary biliary cirrhosis (PBC). However, conventional laboratory assays to detect AMA are dependent on the time-consuming method of immunofluorescence microscopy, a method often plagued by problems of nonspecificity. AMAs react against mitochondrial autoantigens including the E2 components of the pyruvate dehydrogenase complex (PDC-E2), the branched-chain 2-oxo-acid dehydrogenase complex (BCOADC-E2), and the 2-oxo-glutarate dehydrogenase complex (OGDC-E2). Interestingly, the immunodominant epitopes of PDC-E2, BCOADC-E2, and OGDC-E2 are all conformational lipoate binding sites, but antibodies against them do not cross-react. Although 80% to 90% of sera from patients with PBC react to PDC-E2, approximately 10% patients with PBC react only to BCOADC-E2 and/or OGDC-E2. We have taken advantage of our epitope-mapping studies of the E2 components of PDC, BCOADC, and OGDC, and constructed a "designer" hybrid clone, designated as pML-MIT3, that coexpresses the immunodominant epitopes within the three distinct lipoyl domains. We examined a total of 321 sera, including 186 sera from patients with PBC, to test the immunoreactivity of pMIT3. Of 186 sera from patients with PBC, 152 sera (81.7%) reacted with recombinant fusion protein of PDC-E2, whereas 171 sera (91.9%) showed positive reactivities when probed by immunoblotting against the recombinant fusion protein expressed from the pML-MIT3 clone. Of 34 PBC sera that did not react with recombinant PDC-E2, 18 sera contained BCOADC-E2-specific AMA and 1 serum possessed only OGDC-E2-specific AMA. We also developed an enzyme-linked immunosorbent assay (ELISA), using affinity-purified recombinant fusion protein of pML-MIT3 clone as the antigen source, to quantify specific AMAs in patients with PBC. None of the 135 control sera from patients with primary sclerosing cholangitis (PSC), chronic autoimmune hepatitis (CAH), systemic lupus erythematosus (SLE), or healthy volunteers showed significant reactivity against pML-MIT3 recombinant fusion protein in the ELISA assay. Our results indicate that an ELISA using recombinant, cloned autoantigen of pML-MIT3 is a powerful and very specific method for the detection of AMA.

3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)↗
PubMed1996

Effects of ursodeoxycholic acid on survival in patients with primary biliary cirrhosis.

BACKGROUND & AIMS: Ursodeoxycholic acid (UDCA) has been shown to be a safe and effective treatment for patients with primary biliary cirrhosis; however, its effect on patient survival is less certain. To study this issue, the survival of patients receiving long-term UDCA treatment was compared with that of a control group, adjusting for their risk scores based on the Mayo model. METHODS: One hundred eighty patients were randomized to receive either 13-15 mg.kg-1.day-1 UDCA (n = 89) of placebo (n = 91). After the study closure, the patients originally receiving placebo were switched to active drug, and prospective follow-up was continued for 3 years. Patients were censored at the time of transplantation, voluntary withdrawal, of crossover of the placebo group (efficacy analysis). The survival of the two groups was adjusted for risk scores at the time of entry to the study. A secondary analysis was an intent-to-treat analysis, whereby patients were followed up regardless of their voluntary withdrawal or crossover. RESULTS: At the time of analysis, the patients receiving placebo had a significantly increased risk of death and/or requiring transplantation (relative risk, 2.6; P = 0.04) compared with the UDCA-treated patients CONCLUSIONS: UDCA should be considered as a safe, effective, and life-extending treatment for patients with primary biliary cirrhosis.

Adult↗
PubMed1996

Effects of ursodeoxycholic acid on hepatic inflammation and histological stage in patients with primary biliary cirrhosis.

OBJECTIVES: Ursodeoxycholic acid (UDCA) has been reported to be of benefit in the treatment of primary biliary cirrhosis; however, the effects of UDCA on the histological features of primary biliary cirrhosis are uncertain. The goal of this study was to determine the histological effects of 2 yr of treatment with UDCA compared to placebo in a prospective randomized trial of primary biliary cirrhosis. We also sought to correlate the changes in inflammation and histological stage with changes in serum bilirubin, Mayo Risk Score, and the percentage of UDCA in bile in these patients. METHODS: Sixty-one patients (32 receiving UDCA, 29 receiving placebo) who had initial and 2-yr biopsies were studied. Biopsy specimens were evaluated by a single pathologist under coded identification. The degree of inflammation and histological stage were graded on a scale of 0 to 4. RESULTS: There was no significant difference in the degree of inflammation with UDCA treatment when compared to the placebo group at 2 yr. There was a significant but weak indirect association between degree of enrichment of UDCA and changes in inflammation (r = -0.34, p = .02). There was no detectable change in stage in either the UDCA-treated or placebo group when comparing pre- and posttreatment specimens. There were no associations with changes in serum bilirubin or Mayo Risk Score and degree of inflammation. CONCLUSIONS: No significant changes in the degree of inflammation or histological stage were apparent after 2 yr of treatment with UDCA or in the placebo group. A tendency toward improvement in inflammation was noted with greater degrees of biliary UDCA enrichment. Longer term studies will be necessary to determine whether UDCA has a beneficial effect on histological stage.

Bilirubin↗
PubMed1996

Liver transplantation for primary sclerosing cholangitis: impact of risk factors on outcome.

The results of liver transplantation in patients with PSC are excellent and the quality of life is markedly improved. Indeed, liver transplantation is the therapy of choice for patients with end-stage PSC. However, in an age of cost containment, it appears that there are several advantages to offering transplant to patients with PSC a little bit earlier rather than later in the course of their disease. It appears that we can further improve survival, decrease morbidity, decrease blood usage, and avoid the risk of developing a cholangiocarcinoma, which occurs sporadically but not infrequently in the PSC patient. In addition, avoidance of right upper quadrant surgery, such as biliary or shunt surgery, appears to offer several advantages by decreasing resource utilization and possibly decreasing mortality. Although the UNOS selection guidelines recommend transplantation of the sickest patient, there appears to be accumulating evidence that transplantation in patients earlier in the course of their end-stage liver disease may improve survival, decrease morbidity, and also importantly, decrease the cost associated with this expensive procedure. Ideally, we would recommend consideration for liver transplantation all PSC patients who have (1) a Mayo risk score of > 4.8 in whom malignancy is ruled out, (2) cirrhosis and complications of portal hypertension such as variceal bleeding, refractory ascites, or portosystemic encephalopathy, or (3) disabling symptoms such as fatigue, pruritus, or recurrent bacterial cholangitis. We believe that biliary surgery to treat dominant strictures should be avoided and that such strictures should be approached either endoscopically or radiographically, which should include brushings, biopsies, and histology to reasonably exclude the diagnosis of cholangiocarcinoma. Finally, we continue to search for risk factors and for early markers of cholangiocarcinoma so these patients can be identified early and this devastating complication can be avoided by early transplantation.

Cholangitis, Sclerosing↗
PubMed1995

The combination of ursodeoxycholic acid and methotrexate for patients with primary biliary cirrhosis: the results of a pilot study.

Ursodeoxycholic acid (UDCA) and methotrexate (MTX) have both been proposed as treatments for patients with primary biliary cirrhosis (PBC). It has been suggested that a combination of the two drugs may offer advantages over either used separately. In this pilot study, we sought to evaluate the safety and efficacy of this combination for patients with PBC. Thirty-two patients with antimitochondrial antibody positive PBC were prospectively entered into a pilot study and received UDCA, 13 to 15 mg/kg/d, in conjunction with MTX, 0.25 mg/kg/wk, for a period of 2 years. The results of this treatment were compared with those obtained from 180 patients with PBC studied in a placebo-controlled trial of UDCA alone conducted during the same period. Patients in the pilot study and randomized study were comparable with regard to age, gender, and liver biochemistries. The UDCA/MTX-treated patients were of earlier histologic stage and had a lower mean Mayo risk score. During this period, seven patients in the UDCA/MTX group were withdrawn, four for pulmonary toxicity (two who required hospitalization), and one each with mouth ulcer, extreme fatigue, and hair loss. The use of UDCA/MTX was not associated with improvement in symptoms. In the patients receiving UDCA/MTX, biochemical changes were comparable to those of patients receiving UDCA alone but superior to those in the placebo group (P < .05). Histological changes were comparable in all groups at 2 years. Cessation of MTX while UDCA was continued led to no deterioration in liver biochemistries.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Characterisation of patients with a complete biochemical response to ursodeoxycholic acid.

Ursodeoxycholic acid (UDCA) leads to biochemical and clinical improvement in many patients with primary biliary cirrhosis (PBC); although, the response is variable. This study compared UDCA treated patients with complete normalisation of biochemical functions to those without such improvement. Of the 65 patients receiving UDCA, 12 (19%) showed normalisation of liver biochemical functions at two years. The remaining 53 patients showed a less complete response. Mean (SD) alkaline phosphatase and total serum bilirubin values were significantly lower at entry in the patients whose liver biochemistry tests normalised (912 (732) U/l v 1417 (1021) U/l, p = 0.003, and 0.7 (12.1 (5.2) mumol/l v 38.9 (48.5) mumol/l, p = 0.0002, respectively), and percentage of UDCA in biliary bile acid was higher (56.3 (9.5)% v 38.3 (21.1)%, p = 0.03). Patients with biochemically and histologically less severe disease, and greater enrichment of biliary bile with UDCA, are more likely to respond favourably to the drug. The main objective of continued study will be to find out if normal liver biochemical functions can retard disease progression. The association of greater UDCA enrichment with complete biochemical responses suggests that higher doses of UDCA should be evaluated.

Adult↗
PubMed1995

Role of hyperbilirubinemia in the impairment of osteoblast proliferation associated with cholestatic jaundice.

Because the osteoporosis occurring in chronic cholestatic liver disease (CCLD) is associated with decreased bone formation and is reversible by liver transplantation, substances retained in plasma during cholestasis may impair osteoblast function. This hypothesis was tested using a new bioassay that measures plasma mitogenic activity (PMA) for normal human osteoblast-like (hOB) cells. In 29 jaundiced patients, mean PMA was 56.4% (P < 0.001) of that in 29 age- and sex-matched normal subjects, and the decrease in PMA was similar in the 14 with CCLD and the 15 with other causes of jaundice. Bile acids and bilirubin are the two major groups of products retained during cholestasis. The common conjugated bile acids and bilirubin were added to normal human plasma in concentrations simulating those found in patients with CCLD. Various bile salts had no effect on PMA whereas unconjugated bilirubin decreased PMA in a dose-dependent fashion (r = -0.98, P < 0.0001) without affecting cell viability. Relatively selective removal of bilirubin from the plasma by photobleaching normalized the decreased PMA in five jaundiced patients but produced no apparent change in five normal subjects. These data support the hypothesis that hyperbilirubinemia or possibly other photolabile substances impair osteoblast proliferative capacity and thus may play a major role in the pathogenesis of the osteoporosis associated with CCLD.

Bilirubin↗
PubMed1995

Abnormal expression of PDC-E2 on the apical surface of biliary epithelial cells in patients with antimitochondrial antibody-negative primary biliary cirrhosis.

The presence of antimitochondrial antibodies (AMA) is a major criterion for the diagnosis of primary biliary cirrhosis (PBC). Although it is not clear that AMA are involved in the pathogenesis of the disease, the study of these autoantibodies has enabled much information to be accumulated about the specificity of this response. The autoantigens have been identified as components of a functionally related enzyme family, the 2-oxo-acid-dehydrogenase complex. Within this complex, pyruvate dehydrogenase E2 subunit (PDC-E2) has been determined to be the immunodominant autoantigen. Using a panel of mouse monoclonal antibodies and human combinatorial autoantibodies, it has been demonstrated that patients with PBC, but not controls, have an abnormal expression of either PDC-E2 or a cross-reacting molecule in the apical region of biliary epithelium. Others have shown a similar reaction using rabbit sera directed to PDC-E2. Our previous studies have concentrated on AMA-positive patients. In this study, the presence of PDC-E2, class II, immunoglobulin (Ig) A, and B7/BB1 in the bile duct epithelial cells of AMA-positive as well as AMA-negative patients is addressed. Most patients with AMA-negative PBC (seven of nine) react in a fashion similar to AMA-positive patients with intense staining of the apical region of the bile duct epithelial cells of "PDC-E2," increased IgA expression, and little major histocompatibility complex (MHC) class II staining in the early-stage patients. Interestingly, the two AMA-negative patients that did not express PDC-E2 on the apical side of their biliary epithelium had anticentromere antibodies and Sjögren's syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Failure of hepatopulmonary syndrome to resolve after liver transplantation and successful treatment with embolotherapy.

The hepatopulmonary syndrome is an uncommon accompaniment of chronic liver disease. The outcome of this disorder after orthotopic liver transplantation is variable. We describe a patient with the hepatopulmonary syndrome who underwent orthotopic liver transplantation for autoimmune hepatitis. Her platypnea and orthodeoxia failed to improve postoperatively. Pulmonary angiography showed large pulmonary arteriovenous shunts that were successfully treated with coil embolotherapy.

Adult↗