An Unexpected Favourable Evolution of Advanced Stage Cirrhosis.

The elimination of the aetiological factors causing liver injury is an important cornerstone in preventing progression and increasing survival in patients with cirrhosis. The authors present the case of a 63-year-old woman with a history of long-term alcohol abuse and consequent liver cirrhosis. Over the years, the patient presented progressive deterioration with severe malnutrition and had multiple hospital admissions due to decompensated cirrhosis, including refractory ascites, variceal bleeding and an extensive portal vein thrombosis (PVT). Anticoagulant therapy was not initiated due to a high risk of variceal bleeding. She eventually became abstinent, but PVT precluded a liver transplant. Over the following 10 years, her performance status gradually improved, with no new decompensation episodes and liver function normalization, although refractory ascites persisted. Abdominal CT showed spontaneous recanalization of the portal vein and a transjugular intrahepatic portosystemic shunt (TIPS) procedure was performed with gradual improvement of ascites. In this atypical case, an unexpected favourable evolution of advanced stage cirrhosis was observed with long-term improvement in clinical status and liver function, resulting in an estimated 10-year cumulative mortality rate of 99.98% and highlighting the importance of abstinence. Unexpectedly, spontaneous complete repermeabilization of the PVT was also observed, despite its extent and the absence of anticoagulation therapy. LEARNING POINTS: The elimination of the aetiological factors causing liver injury is the cornerstone in preventing decompensation and improving prognosis in patients with cirrhosis.Spontaneous complete recanalization of the portal vein may occur.A transjugular intrahepatic portosystemic shunt (TIPS) is effective in managing some of the major complications of portal hypertension, such as refractory ascites, and provides secondary prophylaxis of oesophageal variceal haemorrhage.

Pacemaker implantation in familial amyloid polyneuropathy: when and for whom?

BACKGROUND: Despite the important role of cardiac pacing in preventing syncope and sudden cardiac death in familial amyloid polyneuropathy (FAP), we lack clear guidelines as to the ideal timing and indications for permanent pacemaker implantation. PURPOSE: The purpose of this study was to evaluate the ideal timing for pacemaker implantation in FAP patients submitted to liver transplantation. METHODS: Retrospective study of 258 FAP patients submitted to liver transplantation between 1992 and 2012. Comparison of three groups: (A) patients without pacemaker (N = 122); (B) patients submitted to pacemaker implantation after liver transplantation, with documented conduction disorders (N = 73); and (C) patients submitted to "prophylactic" pacemaker implantation before transplantation, (N = 73). Patients were followed up for 12.2 ± 6.7 years. RESULTS: The majority of patients (57%) were referred for pacemaker implantation, which occurred before liver transplantation in 50% of cases. Patients who required pacemaker after transplantation presented significantly higher Machado-Joseph Score during pre-transplant evaluation than those who did not require pacemaker (24 ± 10 vs 20 ± 10, p = .025), and also exhibited higher levels of hepatic cytolysis enzymes and hyperbilirubinemia. The most common indication for permanent pacemaker was first degree atrioventricular block, with a mean time between transplantation and pacemaker implantation of 8.7 ± 4.2 years. During long-term follow-up, all-cause mortality was 27% and was lowest in the group submitted to pacemaker implantation only after liver transplantation (p = 0.002). CONCLUSION: The majority of FAP patients submitted to liver transplantation will need a pacemaker at some time of follow-up. However, it seems that there is no benefit in "prophylactic" cardiac pacing before liver transplantation.

Sclerosing Mesenteritis and Disturbance of Glucose Metabolism: A New Relationship? A Case Series.

BACKGROUND: Sclerosing mesenteritis is an idiopathic inflammatory and fibrotic disease that affects the mesentery. It is a rare disease, with the total number of reported cases in the literature ranging from 122 to 300. It mainly affects men in the sixth decade of life, and its etiology remains unknown. Clinical presentation is variable, but it is frequently asymptomatic. Diagnosis is often made by computed tomography (CT) scan, although biopsy may be needed for confirmation. An association between other diseases (e.g., neoplasms) and sclerosing mesenteritis has been described, but the relationship between the latter and glucose changes is not disclosed in the currently available literature. CASE REPORT: Five cases of sclerosing mesenteritis and glucose metabolism disorders (impaired fasting glucose and type 2 diabetes mellitus) were retrospectively collected and analyzed. The mean age was 65 ± 9.3 years, 80% were male, and all patients were white. Three patients were asymptomatic and the other 2 (40%) had non-specific chronic abdominal pain. Blood tests revealed normal inflammatory parameters (mean HbA1c was 6.4% and fasting blood glucose was 140 mg/dL). The diagnosis was made by abdominal CT scan. The 2 symptomatic patients underwent therapy with colchicine 1 mg/day, with clinical improvement. During the mean 43-month follow-up period, there was no symptomatic progression, thereby maintaining the usual benign course of this condition. CONCLUSIONS: Sclerosing mesenteritis has only been described in small series and isolated cases, but its diagnosis is becoming more common due to greater access to diagnostic methods and higher awareness of the disease in the medical community. Furthermore, despite the small sample size, we describe a possible association between glucose metabolism impairment and sclerosing mesenteritis.

More Than Just a Case of Polymyalgia Rheumatica.

Malignant neoplasms are associated with a wide range of paraneoplastic rheumatological syndromes. These can be defined as remote effects of cancer, which are not caused by the tumor and its metastasis. The authors describe the case of an 82-year-old man, who presented with a one-month history of pain, stiffness, and functional limitation of the scapular and pelvic girdles. Blood tests showed raised levels of inflammatory markers. He was diagnosed with Polymyalgia Rheumatica, but the lack of response to corticosteroids led to the suspicion of an alternative diagnosis, like, for example, an occult neoplasm. Although patient evaluation was initially normal, five months later he developed macroscopic haematuria and was diagnosed with bladder and prostate carcinomas. After surgical treatment, the patient fully recovered from his rheumatological syndrome. LEARNING POINTS: In cases when polymyalgia rheumatica exhibits atypical clinical features and/or therapeutic resistance, additional investigation is recommended.Paraneoplastic syndromes can lead to the diagnosis of an underlying neoplasm.