Hepatic Osteodystrophy in Primary Biliary Cirrhosis: Effects of Medical Treatment

Objectives : Osteoporosis is a frequent extrahepatic complication of primary biliary cirrhosis. Although histologically similar to the osteoporosis commonly seen in postmenopausal females, the pathogenesis and management of bone disease in patients with primary biliary cirrhosis is poorly understood. The experience with a subgroup of patients with primary biliary cirrhosis treated with vitamin D, calcium, and estrogen supplementation was reviewed to determine the effects of medical treatment on hepatic osteodystrophy. Methods : The records of 203 women with the diagnosis of primary biliary cirrhosis were reviewed retrospectively for lumbar spine bone mineral density, menopausal status, and supplementation with vitamin D, calcium, and estrogen. Results : The 16 postmenopausal patients treated with estrogen replacement had a statistically significant increase in the lumbar spine bone mineral density at 1 yr (+ 0.014 ± 0.049 vs. -0.03 ± 0.046 g/cm² p < 0.038), without a significant change in the serum bilirubin or alkaline phosphatase. In treated patients, vitamin D and calcium supplementation did not lead to significant improvement in lumbar spine bone mineral density. Conclusions : Calcium and vitamin D supplementation, even in the presence of vitamin D deficiency, do not improve lumbar spine bone mineral density in patients with primary biliary cirrhosis. Estrogen replacement in postmenopausal patients, however, does appear to improve lumbar spine bone mineral density without increasing clinical or biochemical cholestasis, a potential complication reported in animal studies. This study should serve as an impetus for a controlled trial of estrogen replacement in postmenopausal patients with primary biliary cirrhosis.     

Primary Biliary Cirrhosis in a Patient with Multiple Sclerosis

A 47-yr-old patient with primary biliary cirrhosis and multiple sclerosis is reported. Although primary biliary cirrhosis has been associated with numerous other autoimmune disorders, it has not, to the authors' knowledge, previously been reported in association with multiple sclerosis.     

Similarity Between Hepatic Graft-versus-host Disease and Primary Biliary Cirrhosis

Similarities between hepatic graft-versus-host disease (GVHD) and primary biliary cirrhosis (PBC) have been reported recently. To examine this association, we studied 60 patients who underwent allogeneic bone marrow transplantation (BMT) consecutively at a single medical institution.

Among the 60 patients, 12 developed hepatic GVHD after BMT and 48 did not. These two groups were compared with respect to various characteristics seen in PBC, such as autoantibodies, human leukocyte antigen (HLA) status, infection and inflammatory cytokines.

The two groups showed a significant difference in HLA DR status. There was also a significant difference in the febrile period and in cytokine levels between the patients with hepatic GVHD and 12 other patients who had no complications after transplantation.

These findings suggest that hepatic GVHD resembles PBC and that HLA DR features of PBC may also be risk factors for the onset of hepatic GVHD.     

38例原发性胆汁性肝硬化患者的肝功能和血清免疫学变化特征

目的 对原发性胆汁性肝硬化患者的临床特征进行同顾性分析,以提高对该病诊治的认识.方法 分析38例原发性胆汁性肝硬化患者的一般资料、临床表现、生化、免疫学及病理学等改变.结果 本组患者中女性33例,确诊时的平均年龄为57.8±2.2岁.症状以黄疽最为多见(76.3%),其次为皮肤瘙痒(44.7%)和乏力(42.1%),14例患者(36.8%)合并腹水,9例患者(23.7%)食道静脉曲张,8例患者(21.1%)伴随其他自身免疫性疾病,其中5例干燥综合征,3例Graves病.所有患者血清碱性磷酸酶、γ谷氨酰转肽酶及胆红素水平明显升高,分别为523.1±72.6 u/L、759.0±47.1 u/L和290.0±50.8 μmol/L,血清丙氨酸转氨酶(ALT),天冬氨酸转氨酶(AST)水平呈轻至中度升高,分别为101.6±27.1 u/L及197.8±39.6 u/L.患者血清IgM升高(3.9±0.4 g/L),行线粒体抗体检查者76.5%(26/34)阳性.结论 原发性胆汁性肝硬化主要累及中年女性,血清碱性磷酸酶及γ-谷氨酰转肽酶水平升高,抗线粒体抗体阳性,血清IgM升高有助于诊断本病.肝活检病理学检杳有助于本病的进一步确诊及组织学分期.     

Magnetic Compression Duct-to-duct Anastomosis for Biliary Obstruction in a Patient with Living Donor Liver Transplantation

Magnetic compression anastomosis (MCA) is a minimally invasive method of performing choledochocholedochostomy without surgery in patients with biliary stricture or obstruction. We describe a successful case involving magnetic compression duct-to-duct biliary reconstruction in right-lobe living donor liver transplantation (RL-LDLT). Endoscopically, a samarium-cobalt (Sm-Co) rare-earth magnet was placed at the superior site of obstruction via the percutaneous transhepatic biliary drainage route, and another Sm-Co magnet was also placed at the inferior site of obstruction with the aid of an endoscope. MCA techniques enabled complete anastomosis without procedure-related complications. In conclusion, the MCA technique is a revolutionary method of performing choledochocholedochostomy in patients with biliary obstruction after LDLT.     

amilial Primary Biliary Cirrhosis

A 53-year old male and his daughter (38 years old) had primary biliary cirrhosis. The daughter had a high titer of antimitochondrial and other autoantibodies. Nine members, including, four living sibs, mother and two of the daughter's healthy offspring have been examined for liver function, the presence of autoimmune antibodies, cell mediated immunity and histocompatibility antigens. The eldest and second sons, mother and sisters of the affected daughter had multiple autoimmune reactions including antismooth-muscle antibodies and decreased peripheral T lymphocyte but liver tests were normal except in the eldest son. Histocompatibility antigens HL-A9 and HL-B5 were present in both patients and the elder son.
Thus the findings suggest a similar genetic predisposition to immunologic abnormalities among the family members of the patients with primary biliary cirrhosis.     

Primary Biliary Cirrhosis in China

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Hepatic Osteodystrophy Is Common in Patients with Noncholestatic Liver Disease

Background  

Patients with cirrhosis are more prone to develop metabolic bone disease. Scanty literature data are available on osteodystrophy in patients from India with noncholestatic liver diseases.     

Disappearance of Oral Lichen Planus After Liver Transplantation for Primary Biliary Cirrhosis and Immunosuppressive Therapy in a 63-year-Old Japanese Woman

Introduction:

There are few reports concerning association between primary biliary cirrhosis (PBC) and lichen planus. In addition, there is only one report about lichen planus after liver transplantation.

Case Presentation:

We describe a case of oral lichen planus (OLP) accompanied with PBC that resolved following liver transplantation 14 years later. This patient received immunosuppressive drugs after liver transplantation.

Discussion:

The disappearance of OLP might be due to immunosuppressive therapy following liver transplantation. Further observations and studies are necessary to clarify the relationship between OLP and PBC.     

Prevalence of Autoimmune Liver Disease Related Autoantibodies in Chinese Patients with Primary Biliary Cirrhosis

Background  

The prevalence of autoimmune liver disease (AiLD)-related autoantibodies has not been defined in Chinese patients with primary biliary cirrhosis (PBC) and therefore needs to be characterized.     

Hepatic Lymph Nodes as a Follow-up Factor in Primary Biliary Cirrhosis: An Ultrasound Study

Background: The aim of this study was to investigate whether changes in lymph node size in the hepatoduodenal ligament reflect changes in biochemical and histologic markers of cholestasis, hepatocellular damage, and humoral immunoreactivity in patients with primary biliary cirrhosis (PBC). Methods: Twenty-three consecutive patients were examined with repeated liver biopsies, laboratory tests, and ultrasonographic examinations of the hepatoduodenal ligament. The effect of treatment with ursodeoxycholic acid (UDCA) on these factors was also studied. Results: A correlation was found between changes in lymph node size and changes in markers of hepatocellular damage, cholestasis, and humoral immunoreactivity and also with intralobular inflammation. In the UDCA-treated patients the node size decreased slightly during 2 years of treatment, whereas it was unchanged in the control group (p = 0.0528). Conclusions: Changes in the size of the lymph nodes in the hepatoduodenal ligament may reflect inflammation and cholestasis in PBC and thus be a valuable indicator in the follow-up of patients with this disease.