Multiplex neuritis in a patient with autoimmune hepatitis： A case report

INTRODUCTIONAutoimmune hepatitis (AIH) is a chronic autoimmune disease of the liver of unexplained aetiology associated with autoantibodies[1]. Overlapping syndromes with other autoimmune liver diseases, such as primary biliary cirrhosis or primary sclero…     

Holmes-Adie syndrome, autoimmune hepatitis and celiac disease： A case report

A 35-year-old female patient presented with the following symptoms of Holmes-Adie syndrome: photophobia, enlargement of the left pupil unresponsive to light, Achilles areflexia. The pilocarpine test was positive. No tumor or other neurological abnormality was found. She had a 19-year history of autoimmune hepatitis. Flares up were observed following each 3 deliveries. At age of 31 she presented with diarrhea and weight loss. Abdominal tumor was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric multicystic lymphangioma. Simultaneously, celiac disease was diagnosed. Gluten-free diet resulted in a significant improvement of celiac disease, but not of autoimmune hepatitis. Autonomic neuropathy was proven by standard cardiovascular tests. The patient was a homozygous carrier for HLA DQ2 antigen characteristic for celiac disease and heterozygous for HLA DR3 B8 frequent in autoimmune liver diseases. Our novel observation on association of Holmes-Adie syndrome with autoimmune hepatitis and celiac disease is suggestive for a common immunological background for all three entities present in a patient with mesenteric multicystic lymphangioma.     

Development of autoimmune hepatitis type 1 after pulsed methylprednisolone therapy for multiple sclerosis： A case reportDevelopment of autoimmune hepatitis type I after pulsed methylprednisolone therapy for multiple sclerosis： A case report

A 43-year-old woman with multiple sclerosis （MS） was treated with pulsed methylprednisolone and interferon 13 at a hospital. Four weeks after initiating treatment, liver dysfunction occurred and she was referred and admitted to our hospital. Clinical and laboratory findings were consistent with and fulfilled the criteria for drug-induced hepatitis, but not for autoimmune hepatitis （AIH）. She was successfully treated with corticosteroids. As ataxia developed after i year, she was treated with pulsed methylprednisolone for 3 days, then readmitted to our hospital when liver dysfunction occurred. Clinical and laboratory findings led to the diagnosis of AIH. To the best of our knowledge, this is the second case of AIH developed after pulsed methylprednisolone for MS.     

Hepatocellular carcinoma with chronic B-type hepatitis complicated by autoimmune hemolytic anemia： A case report

A 57-year-old man consulted a local hospital because of a persistent slight fever. At the age of 37 years he was diagnosed having B-type hepatitis, but left the liver dysfunction untreated. Twenty years later, he was diagnosed having chronic hepatitis B, hepatocellular carcinoma (HCC) and macrocytic anemia, and referred to our hospital for further investigation. A HCC with a maximum diameter of 5.2 cm was detected in segment 8. Results of blood tests included 1.8 mg/dL serum total bilirubin, 0.9 mg/dL bilirubin, less than 10 mg/dL haptoglobin, 7.9 g/dL hemoglobin, 130 fL MCV, and 14.5% reticulocytes. A bone marrow sample showed erythroid hyperplasia. The direct Coombs test gave a positive result. We diagnosed the anemia as autoimmmune hemolytic anemia (AIHA), for which prednisolone could not be administered due to positivity for HBsAg and HBeAg. After preparation of washed blood cells for later transfusion, the patient underwent systematic resection of segment 8. The cut surface of the resected specimen demonstrated an encapsulated yellow-brownish tumor measuring 52 mm × 40 mmwhich was diagnosed pathologicaly as moderately differentiated HCC. On the 9th postoperative day, the patient's temperature rose to 38℃, and exacerbated hemolysis was observed. The maximum total bilirubin value was 5.8 mg/dL and minimum hemoglobin level was 4.6 g/dL. He tolerated this period without blood transfusion. Currently he is being followed up as an outpatient, and shows no signs of HCC recurrence or symptoms of anemia. AIHA associated with HBV infection has been described in only three previous cases, and the present case is the first in which surgery was performed for accompanying HCC.     

Rectal carcinosarcoma： A case report and review of literature

A 60-years old male was admitted to our department for investigation of constipation and hypogastric discomfort intensified during defecation of a few weeks duration. The cause proved to be a rectal carcinosarcoma that was treated by abdominoperineal resection and postoperative chemo-radiotherapy. The patient died 6 months later due to hepatic failure, showing evidence of disseminated disease. In general colonic carcinosarcomas constitute a rare category of malignant neoplasms whose nature is still incompletely understood. No specific treatment guidelines exist. Surgery is the mainstay of treatment and regardless of the addition of adjuvant therapy the prognosis is very poor. Systematic genetic analysis may be the clue for understanding the pathogenesis of these mysterious tumors.     

Pancreatic metastasis of leiomyosarcoma in the right thigh： A case report

Pancreatic tumors are primary in most of the cases.Pancreatic metastases associated with other primary malignancies,especially pancreatic metastasis of leiomyosarcoma,are uncommon.A 66-year-old woman underwent surgical resection of malignant mesenchymoma(70% osteosarcoma and 30% leiomyosarcoma)in the right thigh.In the postoperative period,a pancreatic mass was identified radiologically by abdominal computed tomography.Pylorus-preserving pancreaticoduodenectomy was performed.The surgical specimen revealed leiomyosarcoma metastasized to the pancreas.A metastatic nodule on the remnant pancreatic tail was discovered 9 mo after the first pancreatic resection,and distal pancreatectomy was performed.Cases of pancreatic metastasis from leiomyosarcoma are extremely rare,especially when the tumor was resectable.We report here a unique case of pancreatic metastasis from a leiomyosarcoma in the right thigh that had been treated surgically.     

Tubulovillous adenoma of anal canal： A case report

Tumors arising from the anal canal are usually of epithelial origin and are mostly squamous cell carcinoma or basal cell carcinoma. We present a case of benign anal adenomas arising from the anus, an extremely rare diagnosis. A 78-year-old white man presented with rectal bleeding of several months duration. Examination revealed a 4 cm friable mass attached to the anus by a stalk. At surgery, the mass was grasped with a Babcock forceps and was resected using electrocautery. Microscopic examination revealed a tubulovillus adenoma with no areas of high grade dysplasia or malignant transformation. The squamocolumnar junction was visible at the edges of the lesion confirming the anal origin of the tumor. We believe the tubulovillus adenoma arose from either an anal gland or its duct that opens into the anus. Although seen rarely, it is important to recognize and treat these tumors at an early stage because of their potential to transform into adenocarcinoma.     

Littoral-cell angioma of the spleen： A case report

Littoral-cell angioma (LCA) is a primary splenic vascular tumor that arises from the normal littoral cells lining the sinus channels of the splenic red pulp. We report a case of LCA of the spleen, which has been infrequently communicated in the literature. A 76-year-old man with a 2-wk history of weight loss, abdominal pain and changes in bowel habits was admitted to our hospital. Imaging studies (CT and MRI) showed multiple lesions in the spleen. Splenectomy was performed. Lining cells were positive for CD31/CD68 markers. Our case was associated with a serrated colonic adenoma. LCA is a benign vascular tumor of the spleen that needs to be included in the differential diagnosis of multiple splenic nodules.     

Concomitant gastric carcinoid and gastrointestinal stromal tumors： A case report

A gastric carcinoid tumor concomitant with gastrointestinal stromal tumor （GIST） is rarely encountered in clinical practice. We report a 65-year-old female who had a 0.8 cm gastric carcinoid tumor on the posterior wall of the upper gastric corpus detected during an esophagogastroduodenoscopy at a routine physical examination, and a concomitant 1.1 cm GIST on the anterior wall of the upper gastric corpus incidentally found during surgery of the gastric carcinoid tumor. Normal serum gastrin level and histological findings suggested that she had a type 111 gastric carcinoid tumor and a GIST which were categorized a very low risk of malignancy, based on their small size and lack of mitosis. Both tumors were treated successfully by surgical excision. The patient had an uneventful recovery. Neither recurrence nor metastasis was found after a 28-mo follow-up.     

自身免疫性胆管炎合并隐匿性乙型肝炎1例

自身免疫性胆管炎临床上少见,合并隐匿性乙型肝炎更为罕见,由于本例患者合并上述两种疾病,治疗上存在矛盾因素,通过综合治疗后取得较好疗效.     

亚急性重型肝炎为首发症状的自身免疫性肝炎2例

一 、临床病例： 病例1，女，13岁，因“乏力、纳差1月余，伴眼黄20d、 烦躁1 d”入院。患者1个月前无明显诱因出现乏力、纳差，20d 前出现眼黄，就诊于当地医院，按“急黄肝”给予保肝、退黄 等治疗，症状无明显缓解。1 d 前患儿乏力加重，伴周身不适、 烦躁不能入睡，收入我院。患者有类风湿性关节炎家族史，否 认肝炎家族史、输血史和损肝药物应用史。查体：烦躁不安，计 算力、定向力下降，皮肤巩膜重度黄染。实验室检查：血常规     

儿童自身免疫性肝炎1例

<正>自身免疫性肝炎(autoimmune hepatitis,AIH)可出现高丙种球蛋白血症和自身抗体阳性,临床表现缺乏特异性。多见于女性,且40岁以后发病率高~([1]),最小发病年龄为6个月~([2-4])。目前,儿童AIH的治疗仍以泼尼松联合或不联合硫唑嘌呤为一线用药~([5-7])。如不治疗,其5 a和10 a生存率分别为50%和10%~([8])。1病例摘要患儿女,5岁5个月,藏族。因"皮肤黄、眼黄、尿黄10     

Development of autoimmune hepatitis type I after pulsed methylprednisolone therapy for multiple sclerosis: A case report

A 43-year-old woman with multiple sclerosis (MS) was treated with pulsed methylprednisolone and interferon β at a hospital.Four weeks after initiating treatment,liver dysfunction occurred and she was referred and admitted to our hospital.Clinical and laboratory findings were consistent with and fulfilled the criteria for drug-induced hepatitis,but not for autoimmune hepatitis (AIH).She was successfully treated with corticosteroids.As ataxia developed after 1 year,she was treated with pulsed methylprednisolone for 3 days,then readmitted to our hospital when liver dysfunction occurred.Clinical and laboratory findings led to the diagnosis of A[H.To the best of our knowledge,this is the second case of AIH developed after pulsed methylprednisolone for MS.     

Pediatric autoimmune hepatitis in a patient who presented with erythema nodosum: a case report

Background

Autoimmune hepatitis (AIH) is a form of chronic hepatitis with unclear causative factors and is characterized by immunological and auto-immunological manifestations. Several extrahepatic manifestations, such as other autoimmune disorders, are associated with AIH. AIH with dermatological conditions as the initial manifestation is rare. We report the case of AIH in which erythema nodosum (EN) was the first manifestation.

Case Presentation

An 8-year-old girl with several persistent dermatological lesions was referred to our hospital several months ago. Her skin had nodular, painful, dry, and erythematous lesions, predominantly on the extensor areas of both the legs, with some erythematous patches on her face. Physical examination revealed that she had hepatosplenomegaly as well. Skin biopsy indicated EN. The results of the laboratory tests showed increased levels of several liver enzymes. The patient''s International Autoimmune Hepatitis Group (IAIHG) score was a definite indicator of AIH. The results of liver biopsy indicated AIH. Other causes of EN and abnormal liver function were ruled out. The only obvious cause of skin lesions was chronic inflammation due to an autoimmune response. The patient was treated for AIH, and her skin lesions along with other signs and symptoms resolved.

Conclusions

AIH can present with protean clinical manifestations, and is thus associated with the risk of delayed diagnosis. Dermatological manifestations, including EN, could indicate a serious disease, and further investigation might be required. AIH should be considered as the possible diagnosis in such cases.     

Juvenile autoimmune hepatitis: Spectrum of the disease

Juvenile autoimmune hepatitis(JAIH) is a progressive inflammatory liver disease, affecting mainly young girls, from infancy to late adolescence, characterized by active liver damage, as shown by high serum activity of aminotransferases, by elevated immunoglobulin G levels, high titers of serum non organ-specific andorgan-specific autoantibodies, and by interface hepatitis on liver biopsy. It is a multifactorial disease of unknown etiology in which environmental factors act as a trigger in genetically predisposed individuals. Two types of JAIH are identified according to the autoan-tibody panel detected at diagnosis: AIH-1, characterized by the presence of anti-smooth muscle antibody and/or antinuclear antibody and AIH-2, by anti-liver-kidney microsomal antibody type 1 and/or by the presence of anti-liver cytosol type 1 antibody. Epidemiological distribution, genetic markers, clinical presentation and pattern of serum cytokines differentiate the two types of AIH suggesting possible pathogenetic mechanisms. The most effective therapy for AIH is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis. The aim of this review is to outline the most significant and peculiar features of JAIH, based largely on our own personal database and on a review of current literature.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus: a case report

A 48-year-old woman was admitted to our hospital because of ascites. Laboratory data indicated the presence of systemic lupus erythematosus (SLE) with nephrotic syndrome and elevated hepatic enzymes. Treatment with prednisolone resulted in a marked clinical improvement in renal and liver dysfunction. Histopathologic analysis of renal and liver tissues showed lupus nephritis and liver cirrhosis, respectively. According to the autoimmune hepatitis scoring system, the patient had both SLE and autoimmune hepatitis.     

Autoimmune hepatitis in a patient with systemic lupus erythematosus: a case report

Abstract

A 48-year-old woman was admitted to our hospital because of ascites. Laboratory data indicated the presence of systemic lupus erythematosus (SLE) with nephrotic syndrome and elevated hepatic enzymes. Treatment with prednisolone resulted in a marked clinical improvement in renal and liver dysfunction. Histopathologic analysis of renal and liver tissues showed lupus nephritis and liver cirrhosis, respectively. According to the autoimmune hepatitis scoring system, the patient had both SLE and autoimmune hepatitis.