Fatal fibrosing cholestatic hepatitis following renal transplantation

A 65-year-old HBsAg positive man developed progressive cholestatic liver enzyme abnormalities with histopathological portoportal septum formation, cholestasis, limited mixed infiltrate and hepatocellular ballooning with a ground glass aspect after renal transplantation. Both clinical and pathological features were characteristic of fibrosing cholestatic hepatitis (FCH), a histological variant of hepatitis-B-virus (HBV) infection with a high mortality rate which affects immunocompromised patients. The diagnosis was made about 9 months after transplantation, after retrospective analysis had shown a postoperative increase in HBV replication. Discontinuation of prednisone treatment and starting antiviral lamivudine therapy reduced HBV DNA load immediately. However due to renal failure caused by hepatorenal syndrome, lamivudine therapy had to be interrupted. The patient died following subacute liver failure with progressive FCH. This case illustrates the importance of early diagnosis and treatment with reduction of immunosuppression and institution of antiviral therapy to prevent progression of FCH in immunocompromised HBsAg positive patients.     

肝脏移植术后受体感染性并发症的防治

目的：探讨儿童同种异体肝脏移植术后各种感染类型的防治特点。方法：2000年3月31日我科成功地进行了1例小儿同种异体背驮式肝移植，受体移植；受体为14岁男童，患先天性Carloli病，曾行两次胆道手术，术前患儿胆汁性肝硬化，复发性胆管炎，肝胆管结石，充血性脾肿大伴脾功亢进，为科末期肝病，结果：术后早期曾出现肺部感染等多器官并发症和急性排斥反应，术后17个月出现慢性排斥反应，经积极抗感染，抗病毒及免疫治疗得以控制，至今已存活2年余。结论：各种感染是儿童肝脏移植的主要并发症，根据药敏选择有效的抗生素控制细菌感染，早期应用更昔洛韦和长期服用FK506的免疫治疗，对巨细胞病毒感染的预防非常有效。     

Occult gastrointestinal bleeding due to a Dieulafoy lesion in the terminal ileum

A 50-year-old man awaiting liver transplantation for primary sclerosing cholangitis developed iron-deficiency anaemia. Repeated occult gastrointestinal bleeding led to an increasing need for blood transfusions. After multiple oesophagogastroduodenoscopies and colonoscopies, videocapsule endoscopy finally demonstrated a polyp-like lesion in the terminal ileum. The lesion had not been detected despite two attempts (oral and anal) at double-balloon enteroscopy and even a peroperative enteroscopy. Only during a second laparotomy, again involving peroperative enteroscopy, a small red lesion was detected and resected 80 cm proximal to the ileocecal valve (Bauhin's valve). Histology revealed a Dieulafoy lesion. Four months later, after normalisation and stabilisation of his haemoglobin level, the patient received a successful liver transplant. If the cause of occult gastrointestinal bleeding in a patient remains unclear despite regular endoscopic procedures, new techniques like videocapsule endoscopy and double-balloon enteroscopy may contribute to identifying the cause. This may lead to an exceptional finding such as a Dieulafoy lesion in the distal ileum.     

Successful elimination of hepatitis C virus after hepatic lobectomy in a patient with hepatocellular carcinoma

Hepatocellular carcinoma develops frequently after chronic hepatitis C and B virus infections. Hepatitis B virus has a direct, while hepatitis C virus an indirect role in hepatocarcinogenesis. THE AIM OF OUR WORK: To demonstrate a very unique and interesting case where after the elimination of early detected duplex hepatocellular carcinoma with a combined therapy of PEG-interferon and ribavirin, hepatitis C virus could be eliminated. CASE PRESENTATION: A 53-year-old male patient had chronic hepatitis C infection in his anamnesis. In 1995 histological examination confirmed cirrhosis in his liver. One year later he was non-responder for conventional interferon therapy. In 2002 CT examination confirmed a process with multiple plexus in the liver. With cytologic proof of hepatocellular carcinoma, a resection of the tumor by left-lobectomy of the liver was carried out. Four years after the operation a one-year PEG-interferon-alfa-2a and ribavirin combined therapy was instituted. The patient became virologically negative. CONCLUSION: In chronic liver diseases carcinoma can develop from multiple center at the same time. PEG-interferon and ribavirin combined therapy can be effective in chronic liver disease, as well as after resection of established hepatocellular carcinoma.     

Zum Stand der Lebendorganspende

In the Federal Republic of Germany the number of living organ donations is increasing. In 2001, 382 (16, 3%) kidney transplantations and 95 (12,5%) liver transplantations have been carried out by using organs or tissue of living donors. Graft survival in kidney transplantation is 91% for living related donation and 89% for living unrelated donation compared to 75% for cadaveric donation after a five year follow-up period. Detailed medical examinations of the donor and the recipient are an absolute prerequisite for organ donation. In Germany, living donation must be performed according to the criteria given in the Transplantation Act.     

Organ donation agency: A discourse analysis of correspondence between donor and organ recipient families

Studies about the psychosocial issues concerning organ donation and transplantation tend to focus on the experiences of donor or recipient families. Little is known about the part played by correspondence exchanged between these two groups; in particular how they perceive the agency of organ donation. This is the first analysis to address the representation of the act of donation from the viewpoint of both donor and recipient families through interrogation of archived correspondence data, using linguistic techniques. The data was drawn from a collection of letters, from four USA organ procurement organisations, exchanged between donor and transplant recipient families. Donor families consistently linguistically ascribed agency and accountability for donation to the person who died, the donor. For the recipient families, on the other hand, the ‘giver’ was mainly implied, ambiguous or ascribed to the donor family.     

Purulent pericarditis following myocardial infarction

A 75-year-old man required prolonged intravenous drug therapy for post-myocardial infarction arrhythmias and developed infection with Staphylococcus aureus at the site of his indwelling cannula. He received prompt antibiotic therapy but was readmitted to hospital five weeks later with a terminal illness which proved at post-mortem to be a staphylococcal pleuropericarditis. We consider that this fatal infection originated at his venous cannula site. The problems of diagnosis are discussed.     

Splenic metastasis of hepatocellular carcinoma

A 76-year-old man, who underwent central bisegmentectomy of the liver, transcatheter arterial chemoembolization, and radiofrequency ablation for chronic hepatitis C virus-related hepatocellular carcinoma (HCC), was found to have a 3 cm mass in the spleen and a 2 cm mass in the liver by computed tomography in January 2003. As both tumors were adjacent, a diagnosis of HCC with splenic infiltration was made. In February 2003, transcatheter arterial chemoembolization and splenic arterial chemo-infusion were performed. However, the splenic tumor increased to 5 cm with slight enhancement on contrast-enhanced computed tomography performed 6 months later, while the hepatic tumor had no enhancement. Limited resection of the liver with splenectomy was performed in October 2003. Macroscopically, the splenic tumor showed infiltrative growth without a capsule while the hepatic tumor showed complete necrosis within its capsule. The splenic tumor was limited to the splenic parenchyma. Histologic examination revealed that the splenic tumor was poorly differentiated HCC, leading to the diagnosis of splenic metastasis. The patient is doing well 17 months after surgery without recurrence. One should perform surgery for splenic metastasis of HCC without hesitation whenever possible.     

Liver transplantation in a HIV-positive patient receiving potent antiretroviral therapy; the first case in The Netherlands

In a 49-year-old woman infected with HIV who was receiving highly-active antiretroviral treatment (HAART), terminal liver failure developed. She also had an acute exacerbation of hepatitis B. She was treated by means of liver transplantation and was in good condition two years later. At that time she was treated with tacrolimus, lamivudine, tenofovir, nelfinavir and hepatitis-B immunoglobulin. HIV-RNA and the DNA of hepatitis-B virus could not be detected, her CD4-count was not abnormal and the liver transplant functioned well. No opportunistic infections had developed. HIV infection has long been considered an absolute contraindication to solid organ transplantation, due to the increased risk of infection and rapid progression to AIDS. With HAART, restoration of immune function is possible. Currently, international experience with liver transplantation for HIV-positive patients that are not infected with hepatitis C has shown promising results. Specifically, the risks of transplant rejection, opportunistic infections and progression to AIDS are not increased. Therefore, criteria have been defined for solid organ transplantation in HIV-positive recipients.     

Quality of life after liver transplantation for hepatitis C infection

Introduction Transplantation is a life-saving option for patients with liver disease. However, recovery is variable. Impairments in physical health, emotional wellbeing and quality of life are reported. Quality of life may be worse after transplantation for hepatitis C virus (HCV) infection. Objective To identify factors that could impact on quality of life after liver transplantation for HCV infection. Methods A qualitative design was used. Data were collected by in-depth interview. Colaizzi’s framework for data analysis was used. Results Varying levels of physical and psychological disability persist for many years after liver transplantation. Participants described living productive and meaningful lives. Many reported a more positive outlook on life since transplantation. Many felt stigmatised by the association of liver disease with alcohol/drug misuse. Participants described the uncertainty of life after transplantation. While all expressed gratitude to the donor, emotional responses to the donation varied. Conclusions Transplant recipients exchange one health state for another. Pre-transplant preparation should encourage realistic expectations of life after transplantation. Before transplantation, potential recipients should be given an opportunity to discuss the donation process. This may reduce the burden of emotional debt experienced by some recipients. Feelings of stigma and future uncertainty may be worse for transplant recipients with HCV. A qualitative approach can provide deeper insight into issues affecting quality of life after transplantation for HCV, and explain some of the ambiguous and contradictory findings of previous quantitative studies.     

A patient with biliary papillomatosis,a rare condition of the biliary system,with fatal outcome

A 21-year-old man was admitted because of upper abdominal pain and cholestasis. Endoscopic retrograde cholangiopancreatography was suggestive of primary sclerosing cholangitis. During follow-up the patient developed symptoms which were not compatible with primary sclerosing cholangitis, i.e. icterus and weight loss. Finally the patient died, almost three years after presentation, because of a metastatic adenocarcinoma which had arisen from biliary papillomatosis. Biliary papillomatosis is characterised by papillary adenomatous proliferation of the bile duct epithelium. It has a high chance of malignant degeneration. The only curative option would have been transplantation of the liver and biliary system, but this ought to have happened at an early stage before malignant degeneration had occurred.     

A young man with acute dilated cardiomyopathy associated with methylphenidate

An 18-year-old obese man with a body mass index of 40, diagnosed with attention-deficit hyperactivity disorder and treated with methylphenidate (Concerta) was acutely admitted to hospital with hypoxia and dyspnoea. On investigation signs of liver-, renal-, and heart-failure were found. Noradrenalin infusion was started. Echocardiography showed dilated left ventricle and an ejection fraction (EF) of 25%. Liver function improved, noradrenalin and dobutamine were tapered, but three days after admission a new echocardiography showed an EF of 10%. The patient was transferred to the National Hospital (Rikshospitalet, Oslo), where intensified treatment including intra aortic balloon pump (IABP) was instituted. Cardiac function improved, and 3 weeks later the IABP was disconnected. EF at this point was 15%. The patient was denied heart transplantation due to various cofactors. The investigation concluded with a probable relationship between his cardiomyopathy and the use of methylphenidate (Concerta).     

HIV transmission through transfusion --- Missouri and Colorado, 2008

Transmission of human immunodeficiency virus (HIV) through transfusion of contaminated blood components was documented in the United States in 1982. Since then, the risk for transfusion-transmitted HIV infection has been almost eliminated by the use of questionnaires to exclude donors at higher risk for HIV infection and the use of highly sensitive laboratory screening tests to identify infected blood donations. The risk for acquiring HIV infection through blood transfusion today is estimated conservatively to be one in 1.5 million, based on 2007-2008 data. This report describes the first U.S. case of transfusion-transmitted HIV infection reported to CDC since 2002. A blood center in Missouri discovered that blood components from a donation in November 2008 tested positive for HIV infection. A lookback investigation determined that this donor had last donated in June 2008, at which time he incorrectly reported no HIV risk factors and his donation tested negative for the presence of HIV. One of the two recipients of blood components from this donation, a patient undergoing kidney transplantation, was found to be HIV infected, and an investigation determined that the patient's infection was acquired from the donor's blood products. Even though such transmissions are rare, health-care providers should consider the possibility of transfusion-transmitted HIV in HIV-infected transfusion recipients with no other risk factors.     

预防动脉吻合狭窄技巧在肾移植术中的应用

目的总结预防移植肾动脉狭窄的手术经验，减少术后动脉狭窄并发症。方法回顾性分析313例肾移植手术，术中通过处理供肾动脉、选择受体动脉、改进动脉吻合术式等手术技巧预防动脉狭窄。结果早期10例患者出现动脉成角狭窄，均在术中得到纠正，晚期1例患者出现血管吻合口近端狭窄。结论采用防动脉狭窄手术技巧利于减少肾移植术后血管并发症。     

Knowledge, attitudes and behavior regarding organ transplantation: the impact of the first transplantation from a brain-dead donor under the organ transplantation act

OBJECTIVE: To investigate knowledge, attitudes and behavior regarding organ transplantation, and to estimate the impact of the first organ transplantation from a brain-dead donor under the Organ Transplantation Act. SUBJECTS AND METHODS: A telephone survey was made using the Random Digit Dialing method in May 1999. People living in the Tokyo Metropolitan area ages 20 years or over were asked about their knowledge, attitudes and behavior regarding organ transplantation, and their opinions on the first organ transplantation conduced in February 1999. The results were compared with an opinion poll made 7 months previously to estimate the impact of this first case. RESULTS: The number of respondents was 489 (response rate: 46.0%). 1) Knowledge of organ transplantation: most people knew that organ donation from brain-dead donors was possible under the act, and that written consent on a form and family member's agreement were necessary before donating. More people knew about donor cards. 2) Organ transplantation: more people were willing to donate their organs when they die, and to agree to donation when a family member died who had wanted to donate his/her organs on their death. In the poll 7 months before, fewer people were willing to donate their organs or to agree to donate their family member's organs after brain death compared with after heart arrest. However, no such difference between brain death and heart arrest was found in this later study, suggesting that the distinction between the two was no longer considered to be so important. 3) Respondents themselves having donor cards constituted 15.2%, showing a marked increase from 7 months earlier. Another 39.7% wished to carry a donor card; the most common reason for not carrying one was they did not know how to acquire it. 4) The first transplantation was considered to have contributed to the increase in knowledge, and a favorable change in attitudes and behavior toward organ transplantation. 5) Respondents who agreed to that organ donation from children aged under 15 should be possible constituted 46.4%, of the to be this not being allowed at the present time. 6) The majority of respondents considered that the brain death was diagnosed properly in the first case and that the donation was made voluntarily, concluding that the transplantation was made fairly. 7) Respondents who considered that the privacy of the donor and the recipients was violated were 46.2% and 36.1%, respectively. The attitude of the mass media toward the first transplantation was criticized by 56.9% of the respondents. 8) Respondents who had a favorable impression of the organ transplantation were 61.9%. Such people are likely to donate their organs, to agree to a family member's donation, and to have a donor card than people who had not gained a favorable impression. The two groups exhibited no difference in their knowledge concerning organ transplantation. CONCLUSIONS: An increase in knowledge and a favorable change in attitudes and behavior regarding organ transplantation was confirmed in this survey, as compared with results 7 months earlier, probably because the first transplantation took place. The majority of the respondents considered that the first transplantation was made fairly, although they criticized the attitude of the mass media invading the privacy of the donor and the recipients. Further studies should be made to facilitate effective delivery of donor cards to people who want to carry them, and to change the rules on organ donation from children.     

Pegloticase for Treatment of Tophaceous Polyarticular Gout

This is a case report of a patient with treatment resistant gout who was prescribed pegloticase and developed a severe reaction. A 30-year-old Hawaiian-Filipino man presented with a nine-year history of gout that progressed from episodic monoarticular arthritis, treated with aspiration and corticosteroid injections, to more aggressive disease with more frequent attacks requiring escalation of therapy. He was treated with systemic corticosteroids, colchicine and nonsteroidal anti-inflammatory drugs, but then required allopurinol. Despite aggressive therapy, the patient continued to have hyperuricemia and tophi developed even after treatment with febuxostat and probenicid. The patient became wheel chair bound due to his pain and, at that point, the decision was made to initiate treatment with pegloticase. The patient initially experienced significant improvement with treatment; however, he soon began to have elevation in his serum uric acid levels and developed a severe reaction during treatment.     

Cyanosis and cirrhosis of liver: hepatopulmonary syndrome

A 66-year-old man with Child-B alcoholic cirrhosis presented with melaena due to hemorrhagic gastritis. Clinical examination revealed cyanosis and clubbing accompanied by severe hypoxaemia without signs of obstructive or restrictive lung disease. Contrast-enhanced echocardiography showed right to left shunt due to intrapulmonary shunting leading to the diagnosis of hepatopulmonary syndrome. Hypoxaemia in patients suffering from chronic liver disease can have different causes but in the presence of cyanosis and clubbing without signs of lung disease the hepatopulmonary syndrome should be suspected. When presence of intrapulmonary shunts has been proven by contrast-enhanced echocardiography, a 100% oxygen test can determine the need for pulmonary angiography and thus determine the indication for interventional therapy or liver transplantation. The patient described was treated supportively with long-term oxygen therapy 6 l/min. Three months later his clinical condition was stable. An attempt to reduce the need for nasal oxygen failed.     

Amatoxin poisoning due to soup from personally picked deathcap mushrooms (Amanita phalloides)

Two patients, a 54-year-old man and a 51-year-old woman, presented with abdominal pain, vomiting and diarrhoea; these symptoms developed 9 and 15 hours, respectively, after consumption of soup from mushrooms that they had picked themselves. As a result of these events, a third patient, a 55-year-old woman with diarrhoea who had also eaten the soup, also presented herself. The first patient recognised deathcap or death angel mushrooms (Amanita phalloides) on a photograph. All three patients were treated for amatoxin poisoning with a combination of high-dose penicillin G, silibinin and acetylcysteine intravenously. The poisoning was later confirmed by the results of urinalysis. The patients were discharged in good condition 8 days later.     

Pediatric liver transplantation

Pediatric liver transplantation has evolved over the last two decades into an effective and widely accepted therapy for infants and children. Currently, these high-risk patients achieve 85 to 90% one-year patient survival and an excellent quality of life. This paper reviews the special features of the pediatric recipient, the surgical innovations developed to be able to offer them a transplant (reduced size, live donor, split, and auxiliary partial transplantation), the most significant issues in anesthetic, immunosuppressive and postoperative care in children, as well as a global picture of the results. Additionally, the experience of the Hospital Infantil de México Federico Gómez is presented, as the largest and most successful series of pediatric liver transplantation in the country, where the first successful live donor liver transplantation and the first simultaneous liver-kidney transplantation in a child were performed.     

Tender growth on toe

A 50-year-old Caucasian man came to the dermatology clinic with a tender growth on his great toe. He first noticed it a year earlier after sustaining trauma to the area. The growth had been getting bigger and had intermittently bled. It had also oozed, so his primary care physician treated it as an infection with topical antibiotics, followed by several courses of oral antibiotics. Despite therapy, the lesion continued to grow and the patient was referred to us. The patient had a 3.4-cm-by-1.8-cm exophytic erythematous nodule on the left lateral great toe. What is your diagnosis? How would you manage this condition?